ANCA相关性血管炎肾损害病理表现件.pptx

ANCA相关性血管炎肾损害病理表现,1,.,P-ANCAPerinuclearPatternAnti-myeloperoxidaseMPO-ANCA,C-ANCACytoplasmicPatternAnti-Proteinase3PR3-ANCA,Anti-NeutrophilCytoplasmicAutoantibodies(ANCA),2,LargeVesselVasculitisGiantCellArteritisTakayasuArteritisMediumVesselVasculitisPolyarteritisNodosaKawasakiDiseaseSmallVesselVasculitisANCA-AssociatedVasculitis（AASV）MicroscopicPolyangiitisGranulomatosiswithPolyangiitis(Wegeners)EosinophilicGranulomatosiswithPolyangiitis(Churg-Strauss)ImmuneComplexVasculitisAnti-GBMDiseaseIgAVasculitis(Henoch-Schnlein)CryoglobulinemicVasculitisHypocomplementemicUrticarialVasculitis(Anti-C1qVasculitis)VariableVesselVasculitis(Cogans,Behcets,etc.)SingleOrganVasculitis(cutaneousSVV,primaryCNSvasculitis,etc.)VasculitisAssociatedwithSystemicDiseases(e.g.Rheumatoid,Lupus,Sarcoid,etc.)VasculitisAssociatedwithProbableEtiologies(e.g.HBV,HCV,drug,cancer,etc.),2011ChapelHillConsensusConferenceVasculitisNomenclature,3,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,4,毛细血管袢纤维素样坏死,5,新月体形成,细胞性新月体,纤维细胞性新月体,6,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,7,小动脉纤维素样坏死,小叶间动脉纤维素样坏死，内膜水肿伴炎症细胞浸润肾间质大量炎症细胞浸润,8,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,9,小管间质病变-炎细胞浸润,10,小管间质病变-炎细胞浸润,11,小管间质病变-炎细胞浸润,12,小管间质病变-坏死性肉芽肿形成,13,免疫荧光-寡免疫性,IgG400,14,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