ACTA ENDOCRINOLOGICA (BUC)

The objective of this review is to summarise what is known about mortality in ACTH dependent Cushing’s disease (CD). Specifically, mortality in other causes of Cushing’s\r\nsyndrome (CS) such as ectopic ACTH syndrome and adrenal adenomas is not considered because of the relative rarity of these causes of CS. Six papers addressed mortality of CD in relation to the background population.\r\nThe overall mortality in CD is increased 2-4 fold, but in patients that achieved early remission of hypercortisolaemia the standardised mortality ratio (SMR) is not different from that of the background population. Those in whom hypercortisolaemia was not controlled had a poor outcome (SMR 4-11), and died prematurely. Persistence of disease, older age at\r\ndiagnosis, and presence of hypertension appear as the main determinants of mortality. However, there are several caveats to these conclusions: 1. The number of patients studied is small and the number of deaths even smaller; 2. On average the patients were young (40yrs old) at diagnosis, their follow-up was 10 yrs, and by the age of 60 the expected number of deaths in the background population is going to be small so that differences in causes of death will be difficult to ascertain; 3. SMR of 1.5 -2.0 for patients in remission may not be statistically significant in the reported studies but could become so with larger numbers followed for a much longer time (30-40 yrs). Notwithstanding these caveats, it is encumbent on us as endocrinologists not only to treat the symptoms and complications of CD but also to ensure that patients are ‘cured’ of their hypercortisolism in order to maximise long-term survival.