ANCA相关性血管炎肾损害病理表现.ppt

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1、ANCA相关性血管炎肾损害病理表现P-ANCAPerinuclearPatternAnti-myeloperoxidaseMPO-ANCAC-ANCACytoplasmicPatternAnti-Proteinase3PR3-ANCAAnti-NeutrophilCytoplasmicAutoantibodies(ANCA)LargeVesselVasculitisGiantCellArteritisTakayasuArteritisMediumVesselVasculitisPolyarteritisNodosaKawasakiDiseaseSm

2、allVesselVasculitisANCA-AssociatedVasculitis（AASV）MicroscopicPolyangiitisGranulomatosiswithPolyangiitis(Wegener’s)EosinophilicGranulomatosiswithPolyangiitis(Churg-Strauss)ImmuneComplexVasculitisAnti-GBMDiseaseIgAVasculitis(Henoch-Schönlein)CryoglobulinemicVasculitisHypocomplem

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

4、HBV,HCV,drug,cancer,etc.)2011ChapelHillConsensusConferenceVasculitisNomenclature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免疫病理和电镜—微量或阴性ANCA相关小血管炎肾损害病理表现毛细血管袢纤维素样坏死新月体形成细胞性新月体纤维细胞性新月体小动脉纤维素样坏死小叶间动脉纤维素样坏死肾间质大量炎症细胞浸润小动脉纤维素样坏死小叶间

5、动脉纤维素样坏死，内膜水肿伴炎症细胞浸润肾间质大量炎症细胞浸润小动脉纤维素样坏死慢性化表现：小叶间动脉管腔内水肿，炎症细胞浸润血管腔内膜和肌层均纤维化小管间质病变-炎细胞浸润小管间质病变-炎细胞浸润小管间质病变-炎细胞浸润小管间质病变-坏死性肉芽肿形成免疫荧光-寡免疫性IgG×400免疫荧光左：纤维素样坏死右：从一个纤维素样坏死病灶蔓延至相邻小新月体Fibrin×400电镜毛细血管袢基底膜断裂电镜血管袢基底膜中断，纤维蛋白从破裂的毛细血管袢漏入右边鲍曼囊腔。