自身免疫性多内分泌腺综合征研究进展

Research progress of autoimmune polyendocrine syndromes

自身免疫性多内分泌腺综合征(APS)是指个体在一生中同时或先后发生2种以上自身免疫性内分泌腺病和非内分泌腺病的一组疾病群。根据发病机制不同,其主要分为两种亚型,APS-Ⅰ与自身免疫调节基因(AIRE)突变有关,而人类白细胞抗原和非人类白细胞抗原的多态性可能参与了APS-Ⅱ的发病机制。通过对该疾病的认识,有助于内分泌医生早期诊断该疾病,提高确诊率。

The autoimmune polyglandular syndromes （APS） refer to a group of syndromes comprising a combination of two or more than two kinds of endocrine and nonendocrine autoimmune diseases in the individuals in life. There are mainly two kinds of APS, which differ in their component diseases and in the immunologic features oft.heir pathogenesis. Type I autoimmune polyglandular syndrome, has a unique pathogenic mechanism owing to mutations in the autoimmune regulator （AIRE） gene, whereas, alleles of human leukocyte antigens （HLAs） and non-HLA genes polymorphism may be involved in the pathogenic mechanism in APS-II. Through the understanding of the disease, it is helpful for the endocrinologists to early diagnose this disease and improve its diagnosis rate.