ARTHRITIS & RHEUMATISM Vol. 46, No. 11, November 2002, pp 2983–2989 DOI 10.1002/art.10589 © 2002, American College of Rheumatology Predictors and Outcomes of Scleroderma Renal Crisis The High-Dose Versus Low-Dose D-Penicillamine in Early Diffuse Systemic Sclerosis Trial Paul J. DeMarco, 1 Michael H. Weisman, 2 James R. Seibold, 3 Daniel E. Furst, 4 Weng Kee Wong, 5 Eric L. Hurwitz, 5 Maureen Mayes, 6 Barbara White, 7 Fredrick Wigley, 8 Walter Barr, 9 Larry Moreland, 10 Thomas A. Medsger, Jr., 11 Virginia Steen, 12 Richard W. Martin, 13 David Collier, 14 Arthur Weinstein, 15 Edward Lally, 16 John Varga, 17 Steven R. Weiner, 4 Brian Andrews, 18 Micha Abeles, 19 and Philip J. Clements 4 Objective. The reported frequency of scleroderma M01-R renal crisis (SRC) in diffuse systemic sclerosis (SSc; scleroderma) is 15–20%. Early use of angiotensin- converting enzyme (ACE) inhibitors has markedly im- proved outcome. The present analysis reexamines the prognostic factors for and outcome of SRC in a prospec- tive cohort of patients with early diffuse SSc. Methods. We retrospectively evaluated the cohort of SSc patients who participated in the High-Dose Versus Low-Dose D-Penicillamine in Early Diffuse SSc trial. Patients with diffuse cutaneous scleroderma were enrolled if their disease duration was <18 months. Because the trial failed to show a difference between treatment groups, the data were pooled. Results. One hundred thirty-four SSc patients entered the observation period a mean  SD of 0.8  0.3 years after onset of SSc. SRC occurred in 18 patients a mean  SD of 0.9  1.1 years after entry. During a mean  SD 4.0  1.1 years of followup after entry, 9 of the 18 patients died (mean  SD 0.6  0.9 years after SRC onset). Baseline characteristics that predicted SRC included a modified Rodnan skin thickness score of >20 (P < 0.01), enlarged cardiac silhouette on radiograph (P  0.04), large joint contractures (wrist, elbow, knee) (P  0.008), and prednisone use at entry (P  0.01). Baseline characteristics that did not predict SRC in- cluded age, sex, race, Health Assessment Questionnaire score, fist closure, handspread, lung involvement, mus- cle weakness, erythrocyte sedimentation rate, and plate- let count. In 5 of 10 subjects for whom at least 2 sequential skin scores were available, skin scores in- creased significantly (P  0.012) in the 6 months before onset of SRC. Conclusion. SRC occurred in 13% of patients soon (mean 11 months) after entry into the cohort. Predictors of SRC identified in this study included Supported in part by grants from the Scleroderma Federation, the United Scleroderma Foundation, the FDA Orphan Drug Program, and the Arthritis Foundation, CRC grants M01-RR-00865 and M01- RR-00827, and bequests from the estates of Winifred Krause, Morris Goldsmith, and Takako Ito. 1 Paul J. DeMarco, MD: Marshfield Clinic, Wausau, Wiscon- sin; 2 Michael H. Weisman, MD: Cedars–Sinai Medical Center, Los Angeles, California; 3 James R. Seibold, MD: University of Medicine and Dentistry of New Jersey–Robert Wood Johnson Medical School, New Brunswick; 4 Daniel E. Furst, MD, Steven R. Weiner, MD, Philip J. Clements, MD, MPH: University of California, Los Angeles, School of Medicine; 5 Weng Kee Wong, PhD, Eric L, Hurwitz, PhD: Univer- sity of California, Los Angeles, School of Public Health; 6 Maureen Mayes, MD, MPH: University of Texas, Houston; 7 Barbara White, MD: University of Maryland School of Medicine, Baltimore; 8 Fredrick Wigley, MD: The Johns Hopkins University, Baltimore, Maryland; 9 Walter Barr, MD: Northwestern University Medical School, Chicago, Illinois; 10 Larry Moreland, MD: University of Alabama at Birming- ham; 11 Thomas A. Medsger, Jr., MD: University of Pittsburgh School of Medicine, Pittsburgh, Pennsylvania; 12 Virginia Steen, MD: George- town University Medical Center, Washington, DC; 13 Richard W. Martin, MD: Michigan State University College of Human Medicine, Grand Rapids; 14 David Collier, MD: University of Colorado Health Sciences Center, Denver; 15 Arthur Weinstein, MD: George Washing- ton University Medical Center, Washington, DC; 16 Edward Lally, MD: Brown University School of Medicine, Roger Williams General Hos- pital, Providence, Rhode Island; 17 John Varga, MD: University of Illinois, Chicago; 18 Brian Andrews, MD, PhD: University of Califor- nia, Irvine; 19 Micha Abeles, MD: University of Connecticut Health Center, Farmington. Address correspondence and reprint requests to Philip J. Clements, MD, MPH, University of California, Los Angeles, School of Medicine, 32-59 Rehabilitation Building, 1000 Veteran Avenue, Los Angeles, CA 90095-1670. E-mail: pclements@mednet.ucla.edu. Submitted for publication January 8, 2002; accepted in re- vised form July 15, 2002. 2983