Scleroderma renal crisis: a retrospective multicentre study on 91 patients and 427 controls

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 o...

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Published in:Rheumatology (Oxford, England) England), 2012-03, Vol.51 (3), p.460-467
Main Authors: Guillevin, Loïc, Bérezné, Alice, Seror, Raphaèle, Teixeira, Luis, Pourrat, Jacques, Mahr, Alfred, Hachulla, Eric, Agard, Christian, Cabane, Jean, Vanhille, Philippe, Harle, Jean-Robert, Deleveaux, Isabelle, Mouthon, Luc
Format: Article
Language:English
Subjects:
Acute Kidney Injury - etiology
Acute Kidney Injury - mortality
Acute Kidney Injury - therapy
Adult
Aged
Angiotensin-Converting Enzyme Inhibitors - therapeutic use
Biological and medical sciences
Case-Control Studies
Combined Modality Therapy
Diseases of the osteoarticular system
Female
Follow-Up Studies
Glucocorticoids - therapeutic use
Humans
Male
Medical sciences
Middle Aged
Prednisone - therapeutic use
Prognosis
Renal Dialysis
Retrospective Studies
Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis
Scleroderma, Systemic - complications
Scleroderma, Systemic - mortality
Scleroderma, Systemic - therapy
Survival Rate
Treatment Outcome
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Summary: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 
ISSN:1462-0324
1462-0332
DOI:10.1093/rheumatology/ker271