ANCA相關(guān)性血管炎腎損害病理表現(xiàn)培訓(xùn)課件

P-ANCAPerinuclearPatternAnti-myeloperoxidaseMPO-ANCAC-ANCACytoplasmicPatternAnti-Proteinase3PR3-ANCAAnti-NeutrophilCytoplasmicAutoantibodies(ANCA)

1ANCA相關(guān)性血管炎腎損害病理表現(xiàn)8/23/2024

LargeVesselVasculitis GiantCellArteritis TakayasuArteritisMediumVesselVasculitis PolyarteritisNodosa KawasakiDiseaseSmallVesselVasculitis ANCA-AssociatedVasculitis（AASV） MicroscopicPolyangiitis GranulomatosiswithPolyangiitis(Wegener’s) EosinophilicGranulomatosiswithPolyangiitis(Churg-Strauss) ImmuneComplexVasculitis Anti-GBMDisease IgAVasculitis(Henoch-Sch?nlein) CryoglobulinemicVasculitis HypocomplementemicUrticarialVasculitis(Anti-C1qVasculitis)VariableVesselVasculitis(Cogan’s,Behcet’s,etc.)SingleOrganVasculitis(cutaneousSVV,primaryCNSvasculitis,etc.)VasculitisAssociatedwithSystemicDiseases(e.g.Rheumatoid,Lupus,Sarcoid,etc.)VasculitisAssociatedwithProbableEtiologies(e.g.HBV,HCV,drug,cancer,etc.)2011ChapelHillConsensusConferenceVasculitisNomenclature2ANCA相關(guān)性血管炎腎損害病理表現(xiàn)8/23/2024光鏡腎小球節(jié)段性毛細(xì)血管袢纖維素樣壞死新月體性腎炎，多新舊不等腎小動(dòng)脈10%-50%可有腎小球外小動(dòng)脈纖維素樣壞死，部分可有中等動(dòng)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)3ANCA相關(guān)性血管炎腎損害病理表現(xiàn)8/23/2024毛細(xì)血管袢纖維素樣壞死4ANCA相關(guān)性血管炎腎損害病理表現(xiàn)8/23/2024新月體形成細(xì)胞性新月體纖維細(xì)胞性新月體5ANCA相關(guān)性血管炎腎損害病理表現(xiàn)8/23/2024小動(dòng)脈纖維素樣壞死小葉間動(dòng)脈纖維素樣壞死腎間質(zhì)大量炎癥細(xì)胞浸潤(rùn)6ANCA相關(guān)性血管炎腎損害病理表現(xiàn)8/23/2024小動(dòng)脈纖維素樣壞死小葉間動(dòng)脈纖維素樣壞死，內(nèi)膜水腫伴炎癥細(xì)胞浸潤(rùn)腎間質(zhì)大量炎癥細(xì)胞浸潤(rùn)7ANCA相關(guān)性血管炎腎損害病理表現(xiàn)8/23/2024小動(dòng)脈纖維素樣壞死慢性化表現(xiàn)：小葉間動(dòng)脈管腔內(nèi)水腫，炎癥細(xì)胞浸潤(rùn)血管腔內(nèi)膜和肌層均纖維化8ANCA相關(guān)性血管炎腎損害病理表現(xiàn)8/23/2024小管間質(zhì)病變-炎細(xì)胞浸潤(rùn)9ANCA相關(guān)性血管炎腎損害病理表現(xiàn)8/23/2024小管間質(zhì)病變-炎細(xì)胞浸潤(rùn)10ANCA相關(guān)性血管炎腎損害病理表現(xiàn)8/23/2024小管間質(zhì)病變-炎細(xì)胞浸潤(rùn)11ANCA相關(guān)性血管炎腎損害病理表現(xiàn)8/23/2024小管間質(zhì)病變-壞死性肉芽腫形成12ANCA相關(guān)性血管炎腎損害病理表現(xiàn)8/23/2024免疫熒光-寡免疫性IgG×40013ANCA相關(guān)性血管炎腎損害病理表現(xiàn)8/23/2024免疫熒光左：纖維素樣壞死右：從一個(gè)纖維素樣壞死病灶蔓延至相鄰小新月體Fibrin×40014ANCA相關(guān)性血管炎腎損害病理表現(xiàn)8/23/2024電鏡毛細(xì)血管袢基底