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Rheumatology (Oxford). 2012 Mar;51(3):460-7. doi: 10.1093/rheumatology/ker271. Epub 2011 Nov 15.

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

Author information

1
Department of Internal Medicine, Hôpital Cochin, Paris Cedex 14, France. loic.guillevin@cch.aphp.fr

Abstract

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.

PMID:
22087012
DOI:
10.1093/rheumatology/ker271
[Indexed for MEDLINE]

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