Autoimmune Addison's disease

• Published in: Baillière's best practice & research. Clinical endocrinology & metabolism Vol. 34; no. 1; p. 101379
• Main Authors: Saverino, Serena, Falorni, Alberto
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Ltd 01.01.2020
• Subjects: 21-hydroxylase antibodies; adrenal crisis; autoimmune polyendocrine syndrome; fludrocortisone; gene polymorphism; hydrocortisone; 21-hydroxylase antibodies; adrenal crisis; gene polymorphism; autoimmune polyendocrine syndrome; hydrocortisone; fludrocortisone
• ISSN: 1521-690X; 1878-1594
• DOI: 10.1016/j.beem.2020.101379

Primary adrenal insufficiency (PAI) occurs in 1/5000–1/7000 individuals in the general population. Autoimmune Addison's disease (AAD) is the major cause of PAI and is a major component of autoimmune polyendocrine syndrome type 1 (APS1) and type 2 (APS2). Presence of 21-hydroxylase autoantibodies (21OHAb) identifies subjects with ongoing clinical or pre-clinical adrenal autoimmunity. AAD requires life-long substitutive therapy with two-three daily doses of hydrocortisone (HC) (15–25 mg/day) or one daily dose of dual-release HC and with fludrocortisone (0.5–2.0 mg/day). The lowest possible HC dose must be identified according to clinical and biochemical parameters to minimize long-term complications that include osteoporosis and cardiovascular and metabolic alterations. Women with AAD have lower fertility and parity as compared to age-matched healthy controls. Patients must be educated to double-triple HC dose in the case of fever or infections and to switch to parenteral HC in the case of vomiting, diarrhoea or acute hypotension.