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1、風(fēng)濕熱同濟(jì)大學(xué)附屬同濟(jì)醫(yī)院兒科魏東概念A(yù)組溶血性鏈球菌心臟炎游走性關(guān)節(jié)炎舞蹈病盤形紅斑和皮下結(jié)節(jié)病因和發(fā)病機(jī)理A組乙型溶血性鏈球菌咽峽炎①咽峽存留時(shí)間②菌株：M血清型、粘液樣型③遺傳學(xué)背景病因和發(fā)病機(jī)理分子模擬①夾膜透明質(zhì)酸：關(guān)節(jié)、滑膜②細(xì)胞壁M及相關(guān)蛋白、N-乙酰葡萄糖胺和鼠李糖：心肌、心瓣膜③細(xì)胞膜脂蛋白：心肌肌膜、丘腦下核、尾狀核FIG.9.Reactivityofantistreptococcal-antimyosinMAbwithhumanmyocardiuminanimmunofluore
2、scenceassay.(Reprintedfromreference173withpermissionfromthepublisher.Copyright1989.?TheAmericanAssociationofImmunologists.)FIG.10.Reactivityofantistreptococcal-antimyosinMAb36.2.2?withthesurfaceorextracellularmatrixofratmyocardialcellsinculture.MAb36.2.
3、2?exhibitscytotoxicityagainstratheartcellsinthepresenceofcomplement.(Reprintedfromreference14withpermissionfromthepublisher.Copyright1997.?TheAmericanAssociationofImmunologists.)病因和發(fā)病機(jī)理自身免疫反應(yīng)免疫復(fù)合物病細(xì)胞免疫反應(yīng)異常①T細(xì)胞反應(yīng)增強(qiáng)、淋巴細(xì)胞母細(xì)胞化和增值反應(yīng)減弱、NK細(xì)胞功能增加②白細(xì)胞移動抑制試驗(yàn)增強(qiáng)③扁桃
4、體單核細(xì)胞反應(yīng)異常FIG.2.HowtheimmunesystemrecognizesgroupAstreptococciandusesopsonizationbycomplementandtype-specificantibodyagainstMproteinoranyothersurfacemoleculecapableofgeneratingopsonicantibody.FcreceptorsshownonmacrophagesbindtotheantibodyFcregion,inducin
5、gphagocytosisandkillingofthestreptococci病因和發(fā)病機(jī)理遺傳背景HLA-B35、DR2、DR4、淋巴細(xì)胞表面標(biāo)記D8/17+毒素Figure2.?PathogeneticpathwayforARFandRHD病理急性滲出期：3~4W增生期：風(fēng)濕小體，3~4MoAschoff細(xì)胞。此細(xì)胞體積大，圓形、多邊形，邊界清楚而不整。胞漿豐富均質(zhì)而微嗜雙色。核大，圓形或卵圓形，核膜清晰，染色質(zhì)集中于中央，橫切面呈梟眼狀，縱切面呈毛蟲狀，稍后則核變的濃染，結(jié)構(gòu)不清。除單核外，亦
6、可見雙核或多核。硬化期：瓣膜受累，持續(xù)3~4月Figure3:?MyocardialAschoffbody–thecellsarelarge,elongated,withlargenuclei;somearemultinucleate在纖維素樣壞死基礎(chǔ)上，出現(xiàn)巨噬細(xì)胞吞噬纖維素樣壞死物所形成的阿少夫細(xì)胞，胞界清而不整齊，略嗜雙色，呈梟眼或毛蟲狀．且有少量淋巴細(xì)胞，漿細(xì)胞Figure2:Aorticvalveshowingactivevalvulitis.Thevalveisslightlythicke
7、nedanddisplayssmallvegetations–"verrucae"Figure8:Stenoticmitralvalveseenfromleftatrium.Bothcommissuresarefused;thecuspsareseverelythickened.Theleftatriumishuge.ThevalveisbothincompetentandstenoticFigure9:Openedstenoticmitralvalveshowingthickeningdistort
8、edcusps,adherentcommissureswithcalcificationandthrombusdeposition,andthickening,fusionandshorteningofchordaetendinaeFigure10:Stenoticmitralvalveseenfromleftatrium,showingfusionofcommissures,thickeningandcalcificationofthecusps臨床表
