Angiotensin converting enzyme inhibitors in treatment of scleroderma renal crisis

Evidence Summaries

Level of Evidence = C
ACE inhibitors may improve survival in scleroderma renal crisis.

A prospective analysis 1 of 108 patients with scleroderma renal crisis (SRC) suggested that therapy with ACE inhibitors improved 1-year survival (15% without and 76% with ACE inhibitors, P < 0.001). However, 24 (44%) of 55 patients with scleroderma renal crisis who were treated with ACE inhibitors died early or required permanent dialysis. Eleven of twenty patients (55%) who survived dialysis more than 3 months and continued to receive therapy with ACE inhibitors were able to discontinue dialysis after 3 to 15 months compared with 0 of 15 dialysis patients who did not receive ACE-inhibitor therapy (P = 0.002).

Another prospective uncontrolled study 2 included 145 patients with SRC receiving ACE inhibitors and 662 patients with scleroderma who did not have renal crisis. Of the patients with SRC, 61% had good outcomes (55 received no dialysis, and 34 received temporary dialysis) and 39% had bad outcomes (permanent dialysis or early death). More than half of the patients who initially required dialysis could discontinue it 3 to 18 months later. Survival of patients in the good outcome group was similar to that of patients with diffuse scleroderma who did not have renal crisis (90% at 5 years and 80% to 85% at 8 years).

EULAR 3 states that despite the lack of RCTs, the experts recommend immediate use of ACE inhibitors in the treatment of scleroderma renal crisis (SRC). ACE inhibitors should be continued long-term as long as there is any chance for additional improvement in kidney function.


1. Steen VD, Costantino JP, Shapiro AP, Medsger TA Jr. Outcome of renal crisis in systemic sclerosis: relation to availability of angiotensin converting enzyme (ACE) inhibitors. Ann Intern Med 1990 Sep 1;113(5):352-7.  [PMID:2382917]
2. Steen VD, Medsger TA Jr. Long-term outcomes of scleroderma renal crisis. Ann Intern Med 2000 Oct 17;133(8):600-3.  [PMID:11033587]
3. Kowal-Bielecka O, Fransen J, Avouac J, et al. Update of EULAR recommendations for the treatment of systemic sclerosis. Ann Rheum Dis 2017;76(8):1327-1339  [PMID:27941129]
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