Objective: To determine the outcome of scleroderma renal crisis before and after the availability of angiotensin converting enzyme (ACE) inhibitors.
Design: Evaluation of a large cohort of patients with systemic sclerosis and renal crisis who were followed prospectively.
Setting: University scleroderma center.
Patients: One hundred and eight patients who had scleroderma renal crisis between 1972 and 1987.
Intervention: ACE inhibitors.
Measurements and main results: Therapy with ACE inhibitors has dramatically improved the survival of patients with scleroderma renal crisis (1-year survival, 15% without and 76% with ACE inhibitors; P less than 0.001). However, 24 (44%) of 55 patients with scleroderma renal crisis who were treated with ACE inhibitors died early or required permanent dialysis. Older age, male sex, an initial serum creatinine level of more than 270 mumol/L, inadequately controlled blood pressure, and congestive heart failure were associated with these poor outcomes, but only older age and congestive heart failure were significant in a multivariate logistic regression analysis. 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).
Conclusions: Patients with systemic sclerosis who develop hypertension should be treated with an ACE inhibitor. Improved survival and successful discontinuation of dialysis are possible when ACE inhibitors are used to treat scleroderma renal crisis.