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- Title
Epidemiology, quality of life and complications of primary adrenal insufficiency: a review.
- Authors
Bensing, Sophie; Hulting, Anna-Lena; Husebye, Eystein S.; Kämpe, Olle; Løvås, Kristian
- Abstract
In this article, we review published studies covering epidemiology, natural course and mortality in primary adrenal insufficiency (PAI) or Addison's disease. Autoimmune PAI is a rare disease with a prevalence of 100-220 per million inhabitants. It occurs as part of an autoimmune polyendocrine syndrome in more than half of the cases. The patients experience impaired quality of life, reduced parity and increased risk of preterm delivery. Following a conventional glucocorticoid replacement regimen leads to a reduction in bone mineral density and an increase in the prevalence of fractures. Registry studies indicate increased mortality, especially evident in patients diagnosed with PAI at a young age and in patients with the rare disease autoimmune polyendocrine syndrome type-1. Most notably, unnecessary deaths still occur because of adrenal crises. All these data imply the need to improve the therapy and care of patients with PAI.
- Subjects
ADRENAL insufficiency; QUALITY of life; ADDISON'S disease; BONE density; THERAPEUTIC use of glucocorticoids; DISEASE complications; THERAPEUTICS
- Publication
European Journal of Endocrinology, 2016, Vol 175, Issue 3, pR107
- ISSN
0804-4643
- Publication type
Article
- DOI
10.1530/EJE-15-1242