| Scleroderma renal crisis: a retrospective multicentre study on 91 patients and 427 controls. | |
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MedLine Citation:
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PMID: 22087012 Owner: NLM Status: Publisher |
Abstract/OtherAbstract:
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Objective. Scleroderma renal crisis (SRC) is a severe manifestation of SSc, whose prognosis remains severe, despite treatment with angiotensin-converting-enzyme inhibitor and dialysis. This study was undertaken to describe SRC characteristics, prognosis and outcome, and evaluate the responsibility of CSs in its occurrence.Methods. Analysis concerned 91 SSc patients with SRC who were compared with 427 non-SRC-SSc patients taken as controls.Results. Among the 91 SRC patients, 71 (78.0%) had high blood pressure, 53 (58.2%) hypertensive encephalopathy and 51 (56.0%) thrombotic microangiopathy; 64 (70.3%) had received CSs before or concomitantly with SRC vs 156 (36.5%) non-SRC-SSc patients (P < 0.001). Treated SRC patients also received more prednisone 29.3 (28.4) vs 3.6 (9.9) mg than controls (P < 0.001). SRC clinical outcomes were poor: 49 (53.8%) patients required dialysis, which was definitive for 38. Thirty-seven (40.7%) SRC patients died vs 10.8% of the controls (P < 0.001). Death was most frequent among dialysed patients who never recovered renal function (22 vs 2) and 13 never-dialysed SRC patients died.Conclusions. Although SRC prognosis has improved markedly, SRC remains a severe manifestation of SSc, despite treatment with angiotensin-converting enzyme inhibitor and dialysis. CSs contributed significantly to SRC occurrence. |
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Authors:
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Loïc Guillevin; Alice Bérezné; Raphaèle Seror; Luis Teixeira; Jacques Pourrat; Alfred Mahr; Eric Hachulla; Christian Agard; Jean Cabane; Philippe Vanhille; Jean-Robert Harle; Isabelle Deleveaux; Luc Mouthon |
Publication Detail:
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Type: JOURNAL ARTICLE Date: 2011-11-15 |
Journal Detail:
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Title: Rheumatology (Oxford, England) Volume: - ISSN: 1462-0332 ISO Abbreviation: - Publication Date: 2011 Nov |
Date Detail:
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Created Date: 2011-11-16 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 100883501 Medline TA: Rheumatology (Oxford) Country: - |
Other Details:
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Languages: ENG Pagination: - Citation Subset: - |
Affiliation:
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Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases: Vasculitis and Systemic Sclerosis, Université Paris Descartes, INSERM U1060, Hôpital Cochin, Assistance Public-Hôpitaux de Paris, Paris, Department of Nephrology and Clinical Immunology, Hôpital de Rangueil, Toulouse, Department of Internal Medicine, National Referral Center for Rare Systemic and Autoimmune Diseases: Systemic Sclerosis, Hôpital Claude-Huriez, Université de Lille, Lille, Department of Internal Medicine A, Hôtel-Dieu, Nantes, Department of Internal Medicine, Hôpital Saint-Antoine, Paris, Department of Internal Medicine, Centre Hospitalier, Valenciennes, Department of Internal Medicine, Hôpital de la Conception, Marseille and Department of Internal Medicine, Hôpital Gabriel-Montpied, Clermont-Ferrand, France. |
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