MANAGEMENT OF ENDOCRINE DISEASE: Epidemiology, quality of life and complications of primary adrenal insufficiency: a review

Eur J Endocrinol. 2016 Sep;175(3):R107-16. doi: 10.1530/EJE-15-1242. Epub 2016 Apr 11.

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.

Publication types

  • Review

MeSH terms

  • Adrenal Insufficiency / complications*
  • Adrenal Insufficiency / drug therapy
  • Adrenal Insufficiency / epidemiology*
  • Disease Management
  • Disease Progression
  • Glucocorticoids / therapeutic use
  • Hormone Replacement Therapy
  • Humans
  • Quality of Life*

Substances

  • Glucocorticoids