儿童天疱疮抗体IgG亚型及与天疱疮抗原的反应性  

Investigation of pemphigus vulgaris-IgG subclasses and their reactivity with desmoglein in pemphigus children

作　　者：张国学[1] 王俊秀[2] 梁骏森 吴志华[1] 

机构地区：[1]广东医学院附属医院,广东湛江524001 [2]中山市陈星海医院,广东中山528415 [3]湛江市第二人民医院,广东湛江524001

出　　处：《中国热带医学》2011年第8期927-929,共3页China Tropical Medicine

基　　金：湛江市科技计划项目(No.2007-2009)

摘　　要：目的观察寻常型天疱疮(Pemphigus vulgaris,PV)患儿和一级亲属血清中PV自身抗体(PV-IgG)亚型及其与桥粒芯糖蛋白(Desmoglein,Dsg)反应性,探讨PV-IgG检测的临床意义和PV的发病机制。方法用间接免疫荧光法(Indirect immunofluorescenceI,IF)检测5例PV患儿、12例一级亲属和15例正常对照者血清PV-IgG,并用免疫印迹法(Western blot,WB)检测PV-IgG亚型及其与Dsg1、Dsg3反应性。结果 PV患儿的PV-IgG阳性率和平均抗体滴度明显高于其一级亲属者(P<0.01),活动期患儿的PV-IgG阳性率和平均抗体滴度也明显高于缓解期(P<0.01);PV患儿中PV-IgG阳性率(80.0%)明显高于一级亲属(25.0%)和正常对照组(0.0%)(P<0.01),PV患儿中Dsg3反应性IgG4水平明显高于一级亲属(P<0.01),且其在活动期病例中水平也明显高于缓解期(P<0.05)。结论寻常型天疱疮的发生与PV-IgG水平有关;Dsg3是PV的主要自身抗原I;gG4是PV的主要致病抗体。Objective To observe the pemphigus vulgaris autoantibody（PV-IgG） subclasses and their reactivity with desmoglein（Dsg） in PV children and their first-degree relatives,and to investigate the clinical significance of PV-Ab detection and the pathogenesis of PV.Methods Serum PV-Ab levels,and PV-IgG subclasses and their reactivity with Dsg1 and Dsg3 in 5 PV children,12 first-degree relatives and 15 healthy individuals were determined by indirect immunofluorescence（IIF） and Western blot（WB）,respectively.Results The frequency and average titer of PV-Ab in PV children were significantly higher than those in the first-degree relatives（P0.001）,and so did between active and recovery stages（P0.01）;The detection rate of PV-IgG in PV children（80.0%） was superior to that in the first-degree relatives（25.0%） and normal controls（0%）（P0.001）;Dsg3-reactive IgG4 level was remarkably higher in PV children and active cases than that in the first-degree relatives and remission cases,respectively（P0.01,P0.05）.Conclusions The upregulation of PV-IgG production is implicated in the pathogenesis of PV;Dsg3 is the major autoantigen;IgG4 is a prerequisite for the pathogensis of PV,whereas IgG1,IgG2 and IgG3 may only take part in the process.

关 键 词：儿童寻常型天疱疮 桥粒芯糖蛋白I GG 间接免疫荧光法 免疫印迹法 

分 类 号：R758.66[医药卫生—皮肤病学与性病学]