Cushings syndrome (CS) is a rare but severe disease associated with an increased mortality especially due to cardiovascular diseases, and associated with various systemic complications, including hypertension, glucose intolerance or diabetes mellitus, dyslipidemia and coagulopathy. Few studies have systematically evaluated the mortality of CS in a large and worldwide population of CS. The MISSION study is a retrospective study aimed at assessing the mortality in a large population of patients with all types of CS followed-up in many clinical centres around the world. This report describes the preliminary results of the study. The total number of patients registered in the database was 5092, (79% females and 21% males), with the following distribution of race: 83% Whites, 9% Oriental, 4% Indians, 3% Hispanics and 1% Blacks. Pituitary tumors (66%) represent the most frequent etiology, followed by adrenal diseases (21%), and ectopic disease (5%); a certain number of patients with non definitive diagnosis were classified as ACTH-dependent diseases (5%), ACTH-independent diseases (2%) and disease of unknown origin (1%). The overall mortality accounts for 7% (356 deaths) with a statistically significant difference between patients with active and with inactive disease (P<0.01) and between males and females (P<0.01). The mortality among patients with pituitary adenoma is 5% (168/3661) and its the same of the patients with adrenal adenoma whereas as expected for patients with adrenal carcinoma and ectopic tumor, the mortality is significantly higher than pituitary and adrenal adenoma, being 43.5% (89/206) and 26% (66/255) respectively. Excluding the unknown causes, and the caused associated with tumor progression (21/356, 6%), severe infections or sepsis represent the most frequent (60/356, 17%), while heart stroke (32/356, 9%) and brain stroke (21/356, 6%) are the second and third causes of death. Hypertension represents the most common complication (72%), followed by dyslipidemia (44%) and glucose intolerance (39%), psychiatric diseases (17%), heart diseases (16%), infections (12%), coagulopathy (12%) and brain diseases (6%). In conclusion, the preliminary results of this study including more than 5000 patients confirms that CS is associated with an high mortality rate due to sepsis and cardiovascular accidents, and associated with cardiovascular, metabolic and infective complications.
30 Apr - 04 May 2011
European Society of Endocrinology