Adrenal insufficiency

• Published in: Nature reviews. Disease primers Vol. 7; no. 1; p. 19
• Main Authors: Hahner, Stefanie, Ross, Richard J., Arlt, Wiebke, Bancos, Irina, Burger-Stritt, Stephanie, Torpy, David J., Husebye, Eystein S., Quinkler, Marcus
• Format: Journal Article
• Language: English
• Published: London Nature Publishing Group UK 11.03.2021; Nature Publishing Group
• Subjects: 692/163/2743; 692/163/2743/1279; 692/698/1460/444; 692/698/1460/6; 692/700/1719; Autoimmune diseases; Cancer Research; Congenital diseases; COVID-19; Diabetes; Disease; Endocrine disorders; Epidemiology; Fertility; Hyperplasia; Internal Medicine; Medical Microbiology; Medicine; Medicine & Public Health; Patient education; Peer review; Pregnancy; Primer; Quality of Life Research; Womens health
• ISSN: 2056-676X; 2056-676X
• DOI: 10.1038/s41572-021-00252-7

Adrenal insufficiency (AI) is a condition characterized by an absolute or relative deficiency of adrenal cortisol production. Primary AI (PAI) is rare and is caused by direct adrenal failure. Secondary AI (SAI) is more frequent and is caused by diseases affecting the pituitary, whereas in tertiary AI (TAI), the hypothalamus is affected. The most prevalent form is TAI owing to exogenous glucocorticoid use. Symptoms of AI are non-specific, often overlooked or misdiagnosed, and are related to the lack of cortisol, adrenal androgen precursors and aldosterone (especially in PAI). Diagnosis is based on measurement of the adrenal corticosteroid hormones, their regulatory peptide hormones and stimulation tests. The goal of therapy is to establish a hormone replacement regimen that closely mimics the physiological diurnal cortisol secretion pattern, tailored to the patient’s daily needs. This Primer provides insights into the epidemiology, mechanisms and management of AI during pregnancy as well as challenges of long-term management. In addition, the importance of identifying life-threatening adrenal emergencies (acute AI and adrenal crisis) is highlighted and strategies for prevention, which include patient education, glucocorticoid emergency cards and injection kits, are described. Adrenal insufficiency (AI) is an endocrine disorder characterized by adrenal hypofunction, resulting in cortisol deficiency. In this Primer, Hahner and colleagues describe the different types of AI, their epidemiology, pathophysiology and diagnosis. Additionally, the authors discuss specific challenges associated with the long-term management of this disorder and strategies to prevent adrenal crises.