Scleroderma at end stage renal disease in the United States: patient characteristics and survival

BACKGROUND: The patient characteristics and mortality associated with scleroderma have not been characterized for a national sample of end stage renal disease (ESRD) patients. METHODS: 364,317 patients in the United States Renal Data System initiated on ESRD therapy between 1 January 1992 and 30 June 1997 with valid causes of ESRD were analyzed in an historical cohort study of scleroderma. RESULTS: Of the study population, 820 (0.22%) had scleroderma. The mean age of patients with scleroderma was 56.38 +/- 13.93 years vs. 60.48 +/- 16.51 years for patients with other causes of ESRD (p<0.01 by Student's t-test). In histogram analysis, there were two age peaks: 45-49 and 65-69. In logistic regression, patients with scleroderma, compared to patients with other causes of ESRD, were significantly more likely to be women, Caucasian, younger, and more likely to have congestive heart failure but less likely to have ischemic heart disease, stroke, and receive predialysis erythropoietin. The unadjusted two-year survival of patients with scleroderma during the study period was 49.3% vs. 63.8% in all other patients (adjusted hazard ratio, 1.96, 95% CI 1.70-2.26, p=0.0001 by Cox Regression). CONCLUSIONS: Among patients with ESRD, the demographics of patients with scleroderma were similar to those of patients with scleroderma in the general population. Patients with scleroderma had decreased survival compared to patients with other causes of ESRD, despite being equally likely to be wait listed and receive renal transplantation adjusted for other factors.