[Prognosis of generalized scleroderma. A retrospective study of 78 cases]

From 1960 to 1984, 78 new patients with progressive systemic sclerosis were followed up: 60 women and 18 men whose ages ranged from 20 to 83 years, with a mean age of 58 years. Twenty nine are known to be dead and 3 were lost of follow-up. Forty six have been followed up to the present time for a mean period of 5 years. The cumulative survival rates were 88 +/- 7 p. 100 at one year, 62,5 +/- 11,5 p. 100 at five years and 50,5 +/- 15 p. 100 at ten years. These figures are significantly different from those found in a matched group from the French general population. Nine features at the time of diagnosis which might influence prognosis were studied. Seven factors apparently have not affected prognosis: sex, age, time elapsed between initial symptom and definitive diagnosis, location of scleroderma, blood pressure, erythrocyte sedimentation rate and creatinine clearance. On the other hand, survival declined significantly faster in the 28 patients with anemia than in the 50 patients without anemia (P less than 0,001). Similarly, the 47 patients with radiological pulmonary involvement or pulmonary function abnormalities were at significantly higher risk for death than the 31 patients without interstitial pulmonary fibrosis. Anemia and pulmonary involvement are predictors of mortality and important prognostic tools in the management of the different drugs that have been recommended for the treatment of patients with systemic sclerosis.