Predictors of survival in systemic sclerosis (Scleroderma)

Abstract

We conducted followup of 264 patients with definite systemic sclerosis (SSc) who were entered into the multicenter Scleroderma Criteria Cooperative Study (SCCS) during 1973–1977. At the end of the study (average 5.2 years of followup), 38% were known to be alive, 50% were dead (68% of these deaths definitely related to SSc), and 12% were lost to followup. Survival analyses of 484 demographic, clinical, and laboratory items recorded at entry into the SCCS (within 2 years of physician diagnosis of SSc) were performed. Survival declined linearly, and the cumulative survival rate was 64 years), reduced renal function (blood urea nitrogen >16 mg/dl), anemia (hemoglobin ≤11 gm/dl), reduced pulmonary diffusing capacity for carbon monoxide (≤50% of predicted), reduced total serum protein level (≤6 gm/dl), and reduced pulmonary reserve (forced vital capacity 14 gm/dl or forced vital capacity 64 years old with a blood urea nitrogen level >16 mg/dl. These results may be useful in predicting individual patients at risk for shortened survival.