Department of Clinical Science and KG Jebsen Center for Autoimmune Disorders, University of Bergen, Bergen, Norway; Department of Medicine, Haukeland University Hospital, Bergen, Norway; Department of Medicine, Karolinska Institutet, Stockholm, Sweden.
Department of Endocrinology, Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK.
Lancet. 2021 Feb 13;397(10274):613-629. doi: 10.1016/S0140-6736(21)00136-7. Epub 2021 Jan 20.
Adrenal insufficiency can arise from a primary adrenal disorder, secondary to adrenocorticotropic hormone deficiency, or by suppression of adrenocorticotropic hormone by exogenous glucocorticoid or opioid medications. Hallmark clinical features are unintentional weight loss, anorexia, postural hypotension, profound fatigue, muscle and abdominal pain, and hyponatraemia. Additionally, patients with primary adrenal insufficiency usually develop skin hyperpigmentation and crave salt. Diagnosis of adrenal insufficiency is usually delayed because the initial presentation is often non-specific; physician awareness must be improved to avoid adrenal crisis. Despite state-of-the-art steroid replacement therapy, reduced quality of life and work capacity, and increased mortality is reported in patients with primary or secondary adrenal insufficiency. Active and repeated patient education on managing adrenal insufficiency, including advice on how to increase medication during intercurrent illness, medical or dental procedures, and profound stress, is required to prevent adrenal crisis, which occurs in about 50% of patients with adrenal insufficiency after diagnosis. It is good practice for physicians to provide patients with a steroid card, parenteral hydrocortisone, and training for parenteral hydrocortisone administration, in case of vomiting or severe illness. New modes of glucocorticoid delivery could improve the quality of life in some patients with adrenal insufficiency, and further advances in oral and parenteral therapy will probably emerge in the next few years.
肾上腺功能不全可由原发性肾上腺疾病引起，也可继发于促肾上腺皮质激素缺乏，或由外源性糖皮质激素或阿片类药物抑制促肾上腺皮质激素引起。典型的临床特征为非故意性体重减轻、厌食、体位性低血压、极度疲劳、肌肉和腹痛以及低钠血症。此外，原发性肾上腺功能不全的患者通常会出现皮肤色素沉着和渴望盐。由于初始表现通常不具特异性，因此肾上腺功能不全的诊断常常被延误；必须提高医生的认识，以避免发生肾上腺危象。尽管采用了最先进的类固醇替代疗法，但原发性或继发性肾上腺功能不全患者的生活质量和工作能力下降，死亡率增加。需要对患者进行积极和反复的肾上腺功能不全管理教育，包括在疾病发作、医疗或牙科程序以及严重压力期间如何增加药物剂量的建议，以预防肾上腺危象，在诊断后约有 50%的肾上腺功能不全患者会发生肾上腺危象。医生为患者提供类固醇卡、静脉用氢化可的松，并对其进行静脉用氢化可的松给药培训，以防发生呕吐或严重疾病，这是良好的做法。新的糖皮质激素给药方式可能会改善一些肾上腺功能不全患者的生活质量，并且在未来几年内可能会出现口服和静脉内治疗方面的进一步进展。
