1型糖尿病与自身免疫性多内分泌腺综合征

摘要

Type 1 diabetes mellitus ( T1DM),arising through a complex interaction of immune,genetic and environmental factors,results from autoimmune destruction of insulin-producing β cells.In up to one third of patients the autoimmune attack is not limited to β cells,but expands into autoimmune polyendocrine syndromes(APS).APS are characterized by functional insufficiency of multiple endocrine organs due to an immunologically mediated destructive process.APS can commonly be divided into three types,including APS type Ⅰ,APS type Ⅱ and immune dysregulation,polyendocrinopathy,enteropathy,X-Linked syndrome (IPEX).Here,we discuss the susceptible factors,clinical manifestation,screening and treatment of APS,with the perspective of the clues they can offer to the pathogenesis and treatment of type 1 diabetes mellitus.%1型糖尿病是由于免疫因素、遗传易感性和环境因素相互作用使产生胰岛素的β细胞受到自身免疫破坏所导致.约1/3的1型糖尿病患者其自身免疫攻击并不局限于β细胞,可发展成为自身免疫性多内分泌腺综合征(APS).APS指由自身免疫反应引起多内分泌腺功能受损为主要表现的临床综合征,可分APS-Ⅰ型、APS-Ⅱ型和X连锁多内分泌腺病肠病伴免疫失调综合征(IPEX).现对APS易感因素,临床表现、筛查及治疗等方面进行综述,并与1型糖尿病比较两者在发病机制及治疗上的相互联系.