National acromegaly registries

Abstract

Acromegaly is a rare disease, associated with multiple organs and systems damage. Thanks to implementation of registries, systematic data gathering, knowledge sharing and standardizing medical practices for optimal care greatly improved. Data concerning 19 national acromegaly registries are available, involving more than 16,000 patients. The weighted mean age of diagnosis is 45.2 years and seems stable over time. A macroadenoma is found in 75% of the cases. The proportion of patients operated (>80%) did not change along time. By contrast, use of radiotherapy has decreased while use of medical therapy increased. If judged on IGF-I levels, acromegaly is controlled in 61.3% of the patients. The disease control rate has improved over time. This is likely due to the development of medical treatment strategies. The collection of data about comorbidities in national registries is much less comprehensive than those about epidemiology, disease control or treatment strategies. The most reported comorbidities are arterial hypertension and diabetes. Data concerning mortality trends are controversial. It seems that cancer has become a leading cause of death in acromegaly patients in the last decade, period in which life expectancy improved, while cardiovascular mortality decreased. In conclusion, acromegaly registries offer a global view of the disease with no "a priori" assumptions. This is of outmost importance, because of the large amount of data and the huge number of associated comorbidities. This will help to establish guidelines for management of this rare disease.

Keywords: acromegaly; epidemiology; mortality; registry.