.,ANCA相關(guān)性血管炎腎損害病理表現(xiàn),.,P-ANCAPerinuclearPatternAnti-myeloperoxidaseMPO-ANCA,C-ANCACytoplasmicPatternAnti-Proteinase3PR3-ANCA,Anti-NeutrophilCytoplasmicAutoantibodies(ANCA),.,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,.,光鏡腎小球節(jié)段性毛細血管袢纖維素樣壞死新月體性腎炎，多新舊不等腎小動脈10%-50%可有腎小球外小動脈纖維素樣壞死，部分可有中等動脈受累腎間質(zhì)-小管炎癥，偶見肉芽腫樣病變偶見髓質(zhì)小管周圍炎免疫病理和電鏡微量或陰性,ANCA相關(guān)小血管炎腎損害病理表現(xiàn),.,毛細血管袢纖維素樣壞死,.,新月體形成,細胞性新月體,纖維細胞性新月體,.,小動脈纖維素樣壞死,小葉間動脈纖維素樣壞死腎間質(zhì)大量炎癥細胞浸潤,.,小動脈纖維素樣壞死,小葉間動脈纖維素樣壞死，內(nèi)膜水腫伴炎癥細胞浸潤腎間質(zhì)大量炎癥細胞浸潤,.,小動脈纖維素樣壞死,慢性化表現(xiàn)：小葉間動脈管腔內(nèi)水腫，炎癥細胞浸潤血管腔內(nèi)膜和肌層均纖維化,.,小管間質(zhì)病變-炎細胞浸潤,.,小管間質(zhì)病變-炎細胞浸潤,.,小管間質(zhì)病變-炎細胞浸潤,.,小管間質(zhì)病變-壞死性肉芽腫形成,.,免疫熒光-寡免疫性,IgG400,.,免疫熒光,左：纖維素樣壞死右：從一個纖維素樣壞死病灶蔓延至相