Long-term outcomes of scleroderma renal crisis

Background: Although scleroderma renal crisis, a complication of systemic s clerosis, can be treated with angiotensin-converting enzyme (ACE) inhibitor s, its long-term outcomes are not known. Objective: To determine outcomes, natural history, and risk factors in pati ents with systemic sclerosis and scleroderma renal crisis. Design: Prospective observational cohort study. Setting: University program specializing in scleroderma. Patients: 145 patients with scleroderma renal crisis who received ACE inhib itors and 662 patients with scleroderma who did not have renal crisis. Measurements: Among patients with renal crisis, the four outcomes studied w ere no dialysis, temporary dialysis, permanent dialysis, and early death. D emographic, clinical, and laboratory data were compared to identify risk fa ctors for specific outcomes. Follow-up was 5 to 10 years. Results: 61% of patients with renal crisis had good outcomes (55 received n o dialysis, and 34 received temporary dialysis); only 4 of these (4%) progr essed to chronic renal failure and permanent dialysis. More than half of th e patients who Initially required dialysis could discontinue it 3 to 18 mon ths later. Survival of patients in the good outcome group was similar to th at of patients with diffuse scleroderma who did not have renal crisis. Some patients (39%) had bad outcomes (permanent dialysis or early death). Conclusions: Renal crisis can be effectively managed when hypertension is a ggressively controlled with ACE inhibitors. Patients should continue taking ACE inhibitors even after beginning dialysis in hopes of discontinuing dia lysis.