Scleroderma Renal Crisis Analysis of Prevalence and Outcome in a Large Italian Series

Abstract

During a 30-year period, 323 SSc patients were admitted to a tertiary center specialized in connective tissue diseases. Among them 13 (4|X%) developed scleroderma renal crisis (SRC). These 13 SRC patients with respect to the remaining SSc patients were older, were more often affected by diffuse cutaneous systemic sclerosis (dcSSc), and had slightly higher blood pressure and more frequent heart involvement at initial presentation. Among the 13 SRC patients, an older age, a shorter disease duration, and a higher peak serum creatinine correlated with a poor outcome.SRC in Italian patients showed a lower prevalence (4|X%) than in many earlier reports. Mean survival in SRC patients was 6 years. This tended to be less than the 16.5 years for all others, but this trend was not statistically significant at least for those admitted in the post-captopril era.From a clinical point of view, the higher prevalence of later SRC in dcSSc patients presenting with mild arterial hypertension and or heart involvement is worth noting. Many patients were asymptomatic and were detected only on a routine visit. Such patients must be carefully followed to detect any early finding of SRC.