Abstract：
Autoimmune polyglandular syndrome (APS) is a rare group of immune-mediated disorders, which are typically, but not exclusively, related to the presence of endocrine abnormalities. APS type 2 is the most common subtype of the syndrome, more often observed in adulthood, with a characteristic clinical triad, which includes adrenal insufficiency, autoimmune thyroiditis and diabetes mellitus type 1. Adrenal insufficiency is an essential and necessary clinical manifestation of the syndrome, as it is observed in 100 % of the cases, while it can be accompanied by hyperchloremic metabolic acidosis. Herein, we present a 23 years-old patient with adrenal insufficiency in the context of autoimmune polyglandular syndrome type 2 with coexisting autoimmune thyroiditis and metabolic acidosis with an increased anion gap attributed to prolonged malnutrition. Additionally, we analyze the main clinical features of adrenal insufficiency, which is a central component of autoimmune polyglandular syndrome; highlight characteristics that differentiate the major APS subtypes.
摘要：
自身免疫性多腺综合征（APS）是一组罕见的免疫介导的疾病，通常是，但不限于此,与内分泌异常有关。APS2型是该综合征最常见的亚型，更常见于成年期，具有特征性的临床三联征，包括肾上腺功能不全,自身免疫性甲状腺炎和1型糖尿病。肾上腺功能不全是该综合征的基本和必要的临床表现，正如在100%的病例中观察到的那样，同时可伴有高氯血症代谢性酸中毒1.在这里，我们介绍了一名23岁的肾上腺功能不全患者,其背景是自身免疫性多腺综合征2型,同时存在自身免疫性甲状腺炎和代谢性酸中毒,由于长期营养不良导致阴离子间隙增加.此外,分析肾上腺功能不全的主要临床特点,这是自身免疫性多腺体综合征的中心组成部分；突出区分主要APS亚型的特征。
