OBJECTIVE Although Cushing's disease is a well documented clinical ent
ity, there is no epidemiological information about it. The present stu
dy tries to obtain this information. DESIGN AND PATIENTS Forty-nine pa
tients affected by Cushing's disease living in Vizcaya (Spain) between
1975 and 1992 were considered for an epidemiological study. RESULTS T
he prevalence of known cases at the end of 1992 was 39.1 per million i
nhabitants. The average incidence of newly diagnosed cases was 2.4 cas
es per million people per year. Cushing's disease was more frequent in
women (n=46) than in men (n=3), with a ratio of 15:1. Diabetes mellit
us and hypertension were observed in 38.7 and 55.1% of patients, respe
ctively. Remission of Cushing's disease was achieved in 36 out of 41 p
atients (87.5%). In general, the mortality was higher than that expect
ed for the control population (standardized mortality ratio, SMR 38, 9
5% confidence interval, Cl 2.5-17.9, P<O.03). Concerning the cause of
death, the SWR of vascular disease was 5 (95% Cl 3.4-48.6, P < 0.05).
Higher age, persistence of hypertension and abnormalities of glucose m
etabolism after treatment, were independent predictors of mortality (m
ultlvariate analyses, P<0.01). CONCLUSIONS Prevalence of Cushing's dis
ease was 39.1 cases/million inhabitants and average incidence was 2.4
cases/million per year. Mortality was elevated, due to vascular diseas
e, associated with higher age, persistence of hypertension and impaire
d glucose metabolism.