同济大学附属同济医院儿科

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1、风湿热同济大学附属同济医院儿科魏东概念A组溶血性链球菌心脏炎游走性关节炎舞蹈病盘形红斑和皮下结节病因和发病机理A组乙型溶血性链球菌咽峡炎①咽峡存留时间②菌株：M血清型、粘液样型③遗传学背景病因和发病机理分子模拟①夹膜透明质酸：关节、滑膜②细胞壁M及相关蛋白、N-乙酰葡萄糖胺和鼠李糖：心肌、心瓣膜③细胞膜脂蛋白：心肌肌膜、丘脑下核、尾状核FIG.9.Reactivityofantistreptococcal-antimyosinMAbwithhumanmyocardiuminanimmunofluore

2、scenceassay.(Reprintedfromreference173withpermissionfromthepublisher.Copyright1989. TheAmericanAssociationofImmunologists.)FIG.10.Reactivityofantistreptococcal-antimyosinMAb36.2.2 withthesurfaceorextracellularmatrixofratmyocardialcellsinculture.MAb36.2.

3、2 exhibitscytotoxicityagainstratheartcellsinthepresenceofcomplement.(Reprintedfromreference14withpermissionfromthepublisher.Copyright1997. TheAmericanAssociationofImmunologists.)病因和发病机理自身免疫反应免疫复合物病细胞免疫反应异常①T细胞反应增强、淋巴细胞母细胞化和增值反应减弱、NK细胞功能增加②白细胞移动抑制试验增强③扁桃

4、体单核细胞反应异常FIG.2.HowtheimmunesystemrecognizesgroupAstreptococciandusesopsonizationbycomplementandtype-specificantibodyagainstMproteinoranyothersurfacemoleculecapableofgeneratingopsonicantibody.FcreceptorsshownonmacrophagesbindtotheantibodyFcregion,inducin

5、gphagocytosisandkillingofthestreptococci病因和发病机理遗传背景HLA-B35、DR2、DR4、淋巴细胞表面标记D8/17+毒素Figure2. PathogeneticpathwayforARFandRHD病理急性渗出期：3~4W增生期：风湿小体，3~4MoAschoff细胞。此细胞体积大，圆形、多边形，边界清楚而不整。胞浆丰富均质而微嗜双色。核大，圆形或卵圆形，核膜清晰，染色质集中于中央，横切面呈枭眼状，纵切面呈毛虫状，稍后则核变的浓染，结构不清。除单核外，亦

6、可见双核或多核。硬化期：瓣膜受累，持续3~4月Figure3: MyocardialAschoffbody–thecellsarelarge,elongated,withlargenuclei;somearemultinucleate在纤维素样坏死基础上，出现巨噬细胞吞噬纤维素样坏死物所形成的阿少夫细胞，胞界清而不整齐，略嗜双色，呈枭眼或毛虫状．且有少量淋巴细胞，浆细胞Figure2:Aorticvalveshowingactivevalvulitis.Thevalveisslightlythicke

7、nedanddisplayssmallvegetations–"verrucae"Figure8:Stenoticmitralvalveseenfromleftatrium.Bothcommissuresarefused;thecuspsareseverelythickened.Theleftatriumishuge.ThevalveisbothincompetentandstenoticFigure9:Openedstenoticmitralvalveshowingthickeningdistort

8、edcusps,adherentcommissureswithcalcificationandthrombusdeposition,andthickening,fusionandshorteningofchordaetendinaeFigure10:Stenoticmitralvalveseenfromleftatrium,showingfusionofcommissures,thickeningandcalcificationofthecusps临床表