【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。更多还原

【Abstract】 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(P<0.001),and so did between active and recovery stages(P<0.01);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%)(P<0.001);Dsg3-reactive IgG4 level was remarkably higher in PV children and active cases than that in the first-degree relatives and remission cases,respectively(P<0.01,P<0.05).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.更多还原