Long-Term Outcomes of Scleroderma Renal Crisis

17 October 2000 | Volume 133 Issue 8 | Pages 600-603

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.

Author and Article Information

From Georgetown University, Washington, D.C., and University of Pittsburgh, Pittsburgh, Pennsylvania.

Acknowledgments: The authors thank Dr. Ray Rault for his involvement and care of many of the patients and Dr. Tom Rakowski for encouragement and review of the manuscript.

Grant Support: In part by the Scleroderma Foundation, Danvers, Massachusetts.

Requests for Single Reprints: Virginia Steen, MD, Georgetown University, 3800 Reservoir Road NW, LL Gorman, Washington, DC 20007; e-mail: steenv@gunet.georgetown.edu.

Current Author Addresses: Dr. Steen: Georgetown University, 3800 Reservoir Road NW, LL Gorman, Washington, DC 20007.

Dr. Medsger: University of Pittsburgh, Department of Medicine, Seventh Floor, BST, Southwing, 3500 Terrace Street, Pittsburgh, PA 15261.

Author Contributions: Conception and design: V.D. Steen, T.A. Medsger.

Analysis and interpretation of the data: V.D. Steen, T.A. Medsger.

Drafting of the article: V.D. Steen.

Critical revision of the article for important intellectual content: V.D. Steen, T.A. Medsger.

Final approval of the article: V.D. Steen, T.A. Medsger.

Provision of study materials or patients: V.D. Steen, T.A. Medsger.

Statistical expertise: V.D. Steen.

Obtaining of funding: V.D. Steen.

Administrative, technical, or logistic support: V.D. Steen.

Collection and assembly of data: V.D. Steen, T.A. Medsger.