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.

肾上腺皮质功能不全 (AI) 是一种以肾上腺皮质醇生成绝对或相对不足为特征的病症。原发性 AI (PAI) 很少见，是由直接肾上腺衰竭引起的。继发性 AI (SAI) 更常见，由影响垂体的疾病引起，而在第三级 AI (TAI) 中，下丘脑受到影响。由于外源性糖皮质激素的使用，最普遍的形式是 TAI。AI 的症状是非特异性的，经常被忽视或误诊，并且与缺乏皮质醇、肾上腺雄激素前体和醛固酮有关（尤其是在 PAI 中）。诊断基于肾上腺皮质类固醇激素的测量、它们的调节肽激素和刺激试验。治疗的目标是建立一种激素替代方案，以密切模仿生理性昼夜皮质醇分泌模式，根据患者的日常需求量身定制。本入门指南提供了对怀孕期间 AI 的流行病学、机制和管理以及长期管理挑战的见解。此外，强调了识别危及生命的肾上腺紧急情况（急性 AI 和肾上腺危象）的重要性，并描述了预防策略，包括患者教育、糖皮质激素急救卡和注射包。