Premature mortality in patients with Addison's disease: a population-based study.
BACKGROUND
The survival rate of patients with primary adrenal insufficiency (Addison's disease) undergoing currently accepted replacement therapy is not known, although well-informed patients are considered to have a normal survival rate. In this study, we evaluated the mortality of patients with Addison's disease in Sweden.


METHODS
A population-based, retrospective, observational study was performed, using the National Swedish Hospital and Cause of Death Registers, covering the period from 1987-2001. After a diagnosis of Addison's disease, each patient was followed until the end of follow-up or death. Mortality was compared with that of the Swedish background population.


RESULTS
We identified 1675 patients (995 women and 680 men) diagnosed with primary adrenal insufficiency. The average follow-up from initial diagnosis was 6.5 yr. Five hundred seven patients died during the study period compared with an expected 199. The risk ratio for all-cause mortality was 2.19 (confidence interval 1.91-2.51) for men and 2.86 (confidence interval 2.54-3.20) for women. The excess mortality in both men and women was attributed to cardiovascular, malignant, and infectious diseases. Concomitant diabetes mellitus was observed in 12% of the patients, but only contributed to the increased mortality to a minor extent.


CONCLUSIONS
Compared with the background population, we observed that the risk ratio for death was more than 2-fold higher in patients with Addison's disease. Cardiovascular, malignant, and infectious diseases were responsible for the higher mortality rate.
Bergthorsdottir
Ragnhildur
R
Department of Endocrinology, gr str 8, Sahlgrenska University Hospital, SE-413 45 Göteborg, Sweden. Ragnhildur.Bergthorsdottir@medic.gu.se
Leonsson-Zachrisson
Maria
M
Odén
Anders
A
Johannsson
Gudmundur
G
D000224:Addison Disease / Q000401:mortality*; D000328:Adult; D015331:Cohort Studies; D015897:Comorbidity; D003920:Diabetes Mellitus / Q000453:epidemiology; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D016017:Odds Ratio; D013548:Sweden / Q000453:epidemiology