原发性小血管炎课件

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1、ANCA相关性小血管炎及其肾损害Anti-NeutrophilCytoplasmicAutoantibodies(ANCA)P-ANCAPerinuclearPatternAnti-myeloperoxidaseMPO-ANCAC-ANCACytoplasmicPatternAnti-Proteinase3PR3-ANCALargeVesselVasculitisGiantCellArteritisTakayasuArteritisMediumVesselVasculitisPolyarteritisNodosaKawasakiDiseaseSm

2、allVesselVasculitisANCA-AssociatedVasculitisMicroscopicPolyangiitisGranulomatosiswithPolyangiitis(Wegener’s)EosinophilicGranulomatosiswithPolyangiitis(Churg-Strauss)ImmuneComplexVasculitisAnti-GBMDiseaseIgAVasculitis(Henoch-Schönlein)CryoglobulinemicVasculitisHypocomplementem

3、icUrticarialVasculitis(Anti-C1qVasculitis)VariableVesselVasculitis(Cogan’s,Behcet’s,etc.)SingleOrganVasculitis(cutaneousSVV,primaryCNSvasculitis,etc.)VasculitisAssociatedwithSystemicDiseases(e.g.Rheumatoid,Lupus,Sarcoid,etc.)VasculitisAssociatedwithProbableEtiologies(e.g.HBV,

4、HCV,drug,cancer,etc.)2011ChapelHillConsensusConferenceVasculitisNomenclatureAortaArteriesArterioleCapillaryVenuleVeinANCAAssociatedVasculitisSmallVesselVasculitisEosinophilicGranulomatosiswithPolyangiitis(Churg-Strauss)ANCAAssociatedVasculitisMPAGPAEGPAAAVSVVPICGNMicroscopicP

5、olyangiitisvasculitiswithnoasthmaorgranulomasGranulomatosiswithPolyangiitis(Wegener’s)granulomasandnoasthmaeosinophilia,asthmaandgranulomasglomerulonephritisPauci-immuneCrescenticGlomerulonephritisANCA（抗嗜中性白细胞胞浆抗体)，为ANCA相关小血管炎(AASV)(国内常称原发性小血管炎)的特异性血清学诊断工具我国小血管炎并不少见，北大医院近5年内诊

6、断ANCA（＋）小血管炎1000余例我国该病漏诊或误诊在基层或落后地区仍相当严重，治疗不规范，应进一步重视ANCA相关性小血管炎的诊断和治疗急性期、早期治疗效果往往是显著的，甚至于戏剧性的概　　　述(二)、ANCA相关性小血管炎主要系指1.韦格纳肉芽肿（Wegener’sgranulomatosis）2.显微镜下型(多)血管炎（microscopicpolyangiitis,MPA）3.嗜酸性粒细胞肉芽肿性(多)血管炎（Churg-Strausssyndrome）：既往国内称变应性肉芽肿性血管炎4.少(寡)免疫复合物新月体性肾炎(pauci-im

7、munecrescenticglomerulonephritis,PICGN)：国内也称为局灶节段坏死性肾炎概　　　述新检测的ANCA阳性小血管炎病例（北京大学肾脏病研究所）一、病理表现免疫病理和电镜—微量或阴性光镜肾小球节段性毛细血管袢纤维素样坏死新月体性肾炎，多新旧不等肾小动脉10%-50%可有肾小球外小动脉纤维素样坏死，部分可有中等动脉受累肾间质-小管炎症，偶见肉芽肿样病变偶见髓质小管周围炎ANCA相关小血管炎及其肾损害免疫荧光局灶节段纤维素样坏死肉芽肿形成新月体形成肾脏小动脉纤维素样坏死肾脏小动脉纤维素样坏死中等动脉纤维素样坏死（肾脏弓状

8、动脉和小叶间动脉）小管间质炎二、临床表现非特异性症状：多发于中老年人，多数人有发热、疲乏、关节肌肉疼痛和体重下降等。脏器受累表现：肾脏受