CAJ | 학술논문

大疱性类天疱疮(BP)是一种自身免疫性表皮下大疱性皮肤病,以C3和(或)IgG在基底膜带线状沉积为特征.研究提示,抗BP180 IgG抗体通过激活补体,可能是诱发大疱形成的主要原因,而自身抗体结合的抗原表位多数包括BP180NC16A结构域.由于不同IgG抗体亚型含量以及激活补体的能力不同,其致病性也不同.免疫荧光和酶联免疫吸附试验提示,IgG1和IgG4为主要的自身反应抗体亚型.体外实验和动物模型均证实,IgG1为主要的致病抗体,其含量与BP的严重程度平行;IgG4具有较弱的活化炎症细胞的致病作用和封闭抗原表位的保护作用.最近的研究提示,抗BP180 IgG抗体可以不依赖激活补体和炎症细胞这两种方式诱导表皮真皮分离.因而关于各IgG亚型的致病能力,特别是IgG4的作用现在仍然不清楚.
대포성류천포창(BP)시일충자신면역성표피하대포성피부병,이C3화(혹)IgG재기저막대선상침적위특정.연구제시,항BP180 IgG항체통과격활보체,가능시유발대포형성적주요원인,이자신항체결합적항원표위다수포괄BP180NC16A결구역.유우불동IgG항체아형함량이급격활보체적능력불동,기치병성야불동.면역형광화매련면역흡부시험제시,IgG1화IgG4위주요적자신반응항체아형.체외실험화동물모형균증실,IgG1위주요적치병항체,기함량여BP적엄중정도평행;IgG4구유교약적활화염증세포적치병작용화봉폐항원표위적보호작용.최근적연구제시,항BP180 IgG항체가이불의뢰격활보체화염증세포저량충방식유도표피진피분리.인이관우각IgG아형적치병능력,특별시IgG4적작용현재잉연불청초.
Bullous pemphigoid (BP) is an autoimmune subepidermal bullous disorder characterized by linear deposition of immunoglobulin G (IgG) and/or C3 along the basement membrane zone.It is supposed that anti-BP180 IgG antibodies play a major role in bulla formation via activation of complement.The BP180NC16A domain has been observed in the majority of antigenic epitopes binding to autoantibodies in BP.The differences in both serum concentrations and the ability to activate complement proteins contribute to the variety in the pathogenicity of different IgG subclasses of autoantibodies.Both immunofluorescence assay and enzyme-linked immunosorbent assay have comfirmed that lgGl and IgG4 are the predominant IgG subclasses of anti-BP180 autoantibodies.Furthermore,in vitro experiment and animal experiment have demonstrated that IgG1 autoantibodies are the primary pathogenic autoantibody,and their serum levels are correlated with disease severity.By contrast,IgG4 may play a weak pathogenic role by slightly activating leucocytes as well as a protective role by blocking the epitopes of pathogenic autoantigens in BP.Latest studies have revealed that anti-BP180 autoantibodies may induce the separation of dermis and epidermis independent of the activation of complement proteins as well as inflammatory cells.The real function of different IgG subclasses of autoantibodies,especially the IgG4 subclass,remains unclear in the pathogenesis of BP.