Epidemiology of Fatal Systemic Sclerosis (Diffuse Scleroderma)

Abstract
Records from 20 hospitals and death certificates for systemic sclerosis yielded 53 cases during the 15-year period 1949 [long dash] 1963. For each case, 2 control death certificates of persons dying from various natural causes excluding accidents and suicides and matched for age, race, sex, and time of death were included. Mortality increased sharply since 1959. The sex and race pattern was similar to that described previously for systemic lupus erythematosus. There were 39 females and 14 males. Mortality in Negro females was 3 times that in females (6. 6[long dash]2.2/million/yr.). The rate for males was about 1/3 that for females and no differences between Negro and white males were observed in the small numbers. Mortality increased with age, at least for whites. Survival time from the 1st recorded diagnosis of scleroderma was approximately equal for males and females; it was significantly shorter for Negroes. No differences were found between cases and controls with respect to birthplace, marital status, or occupation. This study presented the 1st analysis of mortality from systemic sclerosis in a defined population. It confirmed an increased predispositon for females over males and demonstrated an increased risk for Negro females over white females.