title: MANAGEMENT OF ENDOCRINE DISEASE: Epidemiology, quality of life and complications of primary adrenal insufficiency: a review.
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.
authors.affiliation: Department of Molecular Medicine and SurgeryKarolinska Institutet, and Department of Endocrinology, Diabetes and Metabolism, Karolinska University Hospital, Stockholm, Sweden sophie.bensing@ki.se.
authors.forename: Sophie
authors.initials: S
authors.lastname: Bensing
authors.affiliation: Department of Molecular Medicine and SurgeryKarolinska Institutet, Stockholm, Sweden.
authors.forename: Anna-Lena
authors.initials: AL
authors.lastname: Hulting
authors.affiliation: Department of Clinical ScienceUniversity of Bergen, Bergen, Norway Department of MedicineHaukeland University Hospital, Bergen, Norway.
authors.forename: Eystein S
authors.initials: ES
authors.lastname: Husebye
authors.affiliation: Department of Medicine (Solna)Centre for Molecular Medicine, Karolinska Institutet, and Department of Endocrinology, Diabetes and Metabolism, Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Olle
authors.initials: O
authors.lastname: Kämpe
authors.affiliation: Department of Clinical ScienceUniversity of Bergen, Bergen, Norway Department of MedicineHaukeland University Hospital, Bergen, Norway.
authors.forename: Kristian
authors.initials: K
authors.lastname: Løvås
mesh_terms: D000309:Adrenal Insufficiency / Q000150:complications* / Q000188:drug therapy / Q000453:epidemiology*; D019468:Disease Management; D018450:Disease Progression; D005938:Glucocorticoids / Q000627:therapeutic use; D020249:Hormone Replacement Therapy; D006801:Humans; D011788:Quality of Life
chemical_list: D005938:Glucocorticoids