A survival study of patients with scleroderma diagnosed over 30 years (1953-1983): the value of a simple cutaneous classification in the early stages of the disease

Abstract

A longitudinal study was carried out on 177 patients with scleroderma between 1953 and 1983. Patients were classified into 3 types according to the extent of skin sclerosis within one year of presentation: Type 1 with sclerodactyly only; Type 2, skin stiffness proximal to the metacarpophalangeal joints but sparing the trunk; and Type 3, diffuse skin stiffness including the trunk. Ten year survival was 71% in Type 1, 58% in Type 2 and 21% in Type 3. Quality of life was best in Type 1, intermediate in Type 2 and worst in Type 3. Nailfold capillary abnormalities did not discriminate between the types, but anticentromere antibody correlated strongly with Type 1. The simple subdivision of scleroderma based on the early extent of clinical skin involvement has been found to be useful in predicting longterm outcome in this highly variable disease.