Angiotensin Converting Enzyme Inhibitors in Treatment of 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.

References

• 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. [PubMed]
• Steen VD, Medsger TA Jr. Long-term outcomes of scleroderma renal crisis. Ann Intern Med 2000 Oct 17;133(8):600-3. [PubMed]
• 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 [PubMed]