Abstract
All cases of systemic scleroderma in Kumamoto Prefecture were examined epidemiologically and prognostically for two years starting on July 1, 1989. A total of 146 patients, 130 females and 16 males were examined. During the two-year observation period, 4 patients died (the mortality rate was 3.92%). The condition of scleroderma was evaluated in 65 patients during the observation period, and the patients were classified into either progressive, stationary or improving groups. The presence or absence of shortened digits, as well as the presence or absence of the disease overlapping with RA (rheumatoid arthritis) were statistically significantly associated with the prognosis of the three groups of patients, as well as with the worsening or improvement of proximal scleroderma, sclerodactylia, flexion contracture, Raynaud’s phenomenon and diffuse hyperpigmentation.