ANCA相關(guān)性血管炎腎損害病理表現(xiàn)件課件

ANCA相關(guān)性血管炎腎損害

病理表現(xiàn)1.ANCA相關(guān)性血管炎腎損害

病理表現(xiàn)1.P-ANCAPerinuclearPatternAnti-myeloperoxidaseMPO-ANCAC-ANCACytoplasmicPatternAnti-Proteinase3PR3-ANCAAnti-Neutrophil

Cytoplasmic

Autoantibodies(ANCA)

2P-ANCAPerinuclearPatternC-AN

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.)2011ChapelHillConsensusConferenceVasculitisNomenclature3LargeVesselVasculitis2011C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)4光鏡ANCA相關(guān)小血管炎腎損害病理表現(xiàn)4毛細血管袢纖維素樣壞死5毛細血管袢纖維素樣壞死5新月體形成細胞性新月體纖維細胞性新月體6新月體形成細胞性新月體纖維細胞性新月體6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7小動脈纖維素樣壞死小葉間動脈纖維素樣壞死7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8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9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，炎癥細胞小管間質(zhì)病變-炎細胞浸潤10小管間質(zhì)病變-炎細胞浸潤10小管間質(zhì)病變-炎細胞浸潤11小管間質(zhì)病變-炎細胞浸潤11小管間質(zhì)病變-炎細胞浸潤12小管間質(zhì)病變-炎細胞浸潤12小管間質(zhì)病變-壞死性肉芽腫形成13小管間質(zhì)病變-壞死性肉芽腫形成13免疫熒光-寡免疫性IgG×40014免疫熒光-寡免疫性IgG×40014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鄰小新月體Fibrin×40015免疫熒光左：纖維素樣壞死Fibrin×40015電鏡毛細血管袢基底膜斷裂16電鏡毛細血管袢基底膜斷裂16電鏡血管袢基底膜中斷，纖維蛋白從破裂的毛細血管袢漏入右邊鮑曼囊腔。17電鏡血管袢基底膜中斷，纖維蛋白從破裂的毛細血管袢漏入右邊鮑曼ANCA相關(guān)性血管炎腎損害

病理表現(xiàn)18.ANCA相關(guān)性血管炎腎損害

病理表現(xiàn)1.P-ANCAPerinuclearPatternAnti-myeloperoxidaseMPO-ANCAC-ANCACytoplasmicPatternAnti-Proteinase3PR3-ANCAAnti-Neutrophil

Cytoplasmic

Autoantibodies(ANCA)

19P-ANCAPerinuclearPatternC-AN

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.)2011ChapelHillConsensusConferenceVasculitisNomenclature20LargeVesselVasculitis2011C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)21光鏡ANCA相關(guān)小血管炎腎損害病理表現(xiàn)4毛細血管袢纖維素樣壞死22毛細血管袢纖維素樣壞死5新月體形成細胞性新月體纖維細胞性新月體23新月體形成細胞性新月體纖維細胞性新月體6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24小動脈纖維素樣壞死小葉間動脈纖維素樣壞死7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25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26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，炎癥細胞小管間質(zhì)病變-炎細胞浸潤27小管間質(zhì)病變-炎細胞浸潤10小管間質(zhì)病變-炎細胞浸潤28小管間質(zhì)病變-炎細胞浸潤11小管間質(zhì)病變-炎細胞浸潤29小管間質(zhì)病變-炎細胞浸潤12小管間質(zhì)病變-壞死性肉芽腫形成30小管間質(zhì)病變-壞死性肉芽腫形成13免疫熒光-寡免疫性IgG×40031