Scleroderma

Virginia D. Steen, MD, and Thomas A. Medsger Jr., MD
Published: October 17th, 2000

Background: Although scleroderma renal crisis, a complication of systemic sclerosis, can be treated with angiotensin-converting enzyme (ACE) inhibitors, its long-term outcomes are not known.

Objective: To determine outcomes, natural history, and risk factors in patients 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 inhibitors and 662 patients with scleroderma who did not have renal crisis.

Measurements: Among patients with renal crisis, the four outcomes studied were no dialysis, temporary dialysis, permanent dialysis, and early death. Demographic, clinical, and laboratory data were compared to identify risk factors for specific outcomes. Follow-up was 5 to 10 years.

Results: 61% of patients with renal crisis had good outcomes (55 received no dialysis, and 34 received temporary dialysis); only 4 of these (4%) progressed to chronic renal failure and permanent dialysis. 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. Some patients (39%) had bad outcomes (permanent dialysis or early death). Conclusions: Renal crisis can be effectively managed when hypertension is aggressively controlled with ACE inhibitors. Patients should continue taking ACE inhibitors even after beginning dialysis in hopes of discontinuing dialysis.

Scleroderma renal crisis (1) is defined as the new onset of severe hypertension associated with a rapid increase in serum creatinine concentration, microangiopathic hemolytic anemia, or both. Outcomes of this disorder, a once-fatal complication of scleroderma, have dramatically improved with the use of angiotensin-converting enzyme (ACE) inhibitors (1). We examined short- and long-term outcomes
of renal crisis in patients taking these medications...

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