IgA血管炎发病机制及胃肠道表现的研究进展  

Pathogenesis and gastrointestinal manifestations of IgA vasculitis

作　　者：杨雅[1] 许琮[1] 覃华[1] 李德民[1] 赵秋[1] 

机构地区：[1]华中科技大学同济医学院附属同济医院消化内科,湖北省武汉市430030

出　　处：《世界华人消化杂志》2016年第3期390-399,共10页World Chinese Journal of Digestology

基　　金：武汉市科技局应用基础研究基金资助项目;No.2014060101010052~~

摘　　要：IgA血管炎是累及小血管的白细胞脆裂性小血管炎,主要临床表现为与凝血功能无关的皮肤紫癜性病变、胃肠道表现、关节炎或关节疼痛、肾脏病变.病因目前尚不明确,可能与感染、疫苗、药物及肿瘤性疾病等相关.发病机制目前考虑与患者自身遗传易感性、IgA1免疫球蛋白"O"铰链区糖基化异常、补体系统异常激活、炎性因子与促炎性因子和自身抗体的产生等方面密切相关.IgA血管炎病理学上典型表现为多形核白细胞渗出及白细胞脆裂性血管炎.IgA血管炎治疗分为对症支持治疗、免疫抑制治疗(糖皮质激素和免疫抑制剂)、血液净化治疗及外科手术治疗.IgA血管炎预后与发病年龄及是否累及肾脏相关.Immunoglobulin A（IgA） vasculitis is a form of leukocytoclastic vasculitis of small vessels, clinically characterized by purpuric skin lesions unrelated to any underlying coagulopathy, gastrointestinal manifestations, arthritis and/or arthralgia and renal involvement. The etiology remains unknown, but various triggers including infections, vaccination, drugs and malignancy have been hypothesized to be associated with the development of IgA vasculitis. Although the pathogenesis has not been completely figured out, genetic predisposition, aberrant glycosylation of the hinge region of IgA1, activated complements, cytokines and chemokines were put out to play important roles in the immunopathogenesis of IgA vasculitis. Histologically, the infiltration of small blood vessels with polymorphonuclear leukocytes and the presence of leukocytoclasia are typical pathologic findings in IgA vasculitis. The treatment is usually supportive, and advanced treatments include immunosuppressive drugs（glucocorticoids and immunosuppressive agents）, hemopurification and surgery. The prognosis depends on the age at the disease onset and the renal involvement or not.

关 键 词：IgA血管炎 腹型 发病机制 内镜检查 治疗 

分 类 号：R593.2[医药卫生—内科学] R57[医药卫生—临床医学]