Endocrine Unit, Department of Medicine (DIMED), University of Padova, Via Ospedale Civile 105, 35128, Padua, Italy.
Unit of Internal Medicine, Ospedale dell'Angelo, via Paccagnella 11, 30174, Mestre-Venice, Italy.
J Endocrinol Invest. 2019 Dec;42(12):1407-1433. doi: 10.1007/s40618-019-01079-6. Epub 2019 Jul 18.
Addison's disease (AD) is a rare disorder and among adult population in developed countries is most commonly caused by autoimmunity. In contrast, in children genetic causes are responsible for AD in the majority of patients.
This review describes epidemiology, pathogenesis, genetics, natural history, clinical manifestations, immunological markers and diagnostic strategies in patients with AD. Standard care treatments including the management of patients during pregnancy and adrenal crises consistent with the recent consensus statement of the European Consortium and the Endocrine Society Clinical Practice Guideline are described. In addition, emerging therapies designed to improve the quality of life and new strategies to modify the natural history of autoimmune AD are discussed.
Progress in optimizing replacement therapy for patients with AD has allowed the patients to lead a normal life. However, continuous education of patients and health care professionals of ever-present danger of adrenal crisis is essential to save lives of patients with AD.
艾迪生病（Addison's disease，AD）是一种罕见疾病，在发达国家的成年人群体中，最常见的病因是自身免疫。相比之下，在儿童中，AD 大多数情况下是由遗传因素引起的。
本综述描述了 AD 患者的流行病学、发病机制、遗传学、自然病史、临床表现、免疫学标志物和诊断策略。描述了 AD 患者的标准治疗方法，包括妊娠期间和与近期欧洲联合会和内分泌学会临床实践指南的共识声明一致的肾上腺危象期间的管理。此外，还讨论了旨在提高生活质量的新兴疗法以及改变自身免疫性 AD 自然病史的新策略。
优化 AD 患者替代疗法的进展使患者能够过上正常的生活。然而，对 AD 患者和医疗保健专业人员进行持续的教育，了解始终存在的肾上腺危象危险，对于挽救 AD 患者的生命至关重要。
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