自身免疫性多内分泌腺病综合征研究进展

Advances in Autoimmune Polyendocrine Syndromes

自身免疫性多内分泌腺病综合征为一类少见的由多个系统免疫损伤引起的临床疾病,主要有三种分型:自身免疫性多内分泌腺病综合征1型(APS-1)、自身免疫性多内分泌腺病综合征2型(APS-2)以及X连锁免疫调节、多内分泌疾病和肠病(IPEX)。免疫与遗传机制均参与了疾病的发生与发展。本文对该类疾病发病机制及治疗的新进展进行综述。

The autoimmune polyendocrine syndromes(APS) are a heterogeneous group of rare diseases characterized by the autoimmune destruction of several organ systems, including three major types: APS type 1(APS-1), APS type 2(APS-2) and Immune dysregulation, polyendocrinopathy, enteropathy, and X-linked(IPEX) disease. Immune and genetic mechanisms are involved in the pathogenesis and progression of the disease. Advances in pathogenesis and treatment of these diseases are discussed in this paper.