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)P-ANCA Perinuclear PatternAnti-myeloperoxidaseMPO-ANCAC-ANCA Cytoplasmic PatternAnti-Proteinase 3PR3-ANCAAnti-Neutrophil Cytoplasmic Autoantibodies (ANCA) Large Vessel VasculitisGiant Cell ArteritisTakayasu ArteritisMedium Vessel VasculitisPolyarteritis NodosaKawasaki DiseaseSmall V

2、essel VasculitisANCA-Associated Vasculitis（AASV）Microscopic PolyangiitisGranulomatosis with Polyangiitis (Wegeners)Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss)Immune Complex VasculitisAnti-GBM DiseaseIgA Vasculitis (Henoch-Schnlein)Cryoglobulinemic VasculitisHypocomplementemic Urtic

3、arial Vasculitis (Anti-C1q Vasculitis)Variable Vessel Vasculitis (Cogans, Behcets, etc.)Single Organ Vasculitis (cutaneous SVV, primary CNS vasculitis, etc.)Vasculitis Associated with Systemic Diseases (e.g. Rheumatoid, Lupus, Sarcoid, etc.)Vasculitis Associated with Probable Etiologies (e.g. HBV, H

4、CV, drug, cancer, etc.) 2011 Chapel Hill Consensus Conference Vasculitis Nomenclature光鏡光鏡 腎小球腎小球節(jié)段性毛細(xì)血管袢纖維素樣壞死節(jié)段性毛細(xì)血管袢纖維素樣壞死新月體性腎炎，多新舊不等新月體性腎炎，多新舊不等 腎小動(dòng)脈腎小動(dòng)脈10%-50%10%-50%可有腎小球外小動(dòng)脈纖維素樣壞死，部分可可有腎小球外小動(dòng)脈纖維素樣壞死，部分可有中等動(dòng)脈受累有中等動(dòng)脈受累 腎間質(zhì)腎間質(zhì)- -小管小管炎癥，偶見肉芽腫樣病變炎癥，偶見肉芽腫樣病變偶見髓質(zhì)小管周圍炎偶見髓質(zhì)小管周圍炎免疫病理和電鏡免疫病理和電鏡微量或陰性微量或陰

5、性ANCA相關(guān)小血管炎腎損害病理表現(xiàn)毛細(xì)血管袢纖維素樣壞死新月體形成細(xì)胞性細(xì)胞性新月體新月體纖維細(xì)胞纖維細(xì)胞性性新月體新月體小動(dòng)脈纖維素樣壞死小葉間動(dòng)脈纖維素樣小葉間動(dòng)脈纖維素樣壞死壞死腎腎間質(zhì)大量炎癥細(xì)胞浸潤間質(zhì)大量炎癥細(xì)胞浸潤小動(dòng)脈纖維素樣壞死小葉間動(dòng)脈纖維素樣壞死小葉間動(dòng)脈纖維素樣壞死，內(nèi)膜內(nèi)膜水腫伴炎癥水腫伴炎癥細(xì)胞細(xì)胞浸潤浸潤腎腎間質(zhì)大量炎癥細(xì)胞浸潤間質(zhì)大量炎癥細(xì)胞浸潤小動(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 血管血管腔內(nèi)膜和肌層均纖維化腔內(nèi)膜和肌層均纖維化小管間質(zhì)病變-炎細(xì)胞浸潤小管間質(zhì)病變-炎細(xì)胞浸潤小管間質(zhì)病變-炎細(xì)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：纖維素樣左：纖維素樣壞死壞死右：從一個(gè)纖維素樣壞死病灶蔓延至相鄰小右：從一個(gè)纖維素樣壞死病灶蔓延至相鄰小新月體新月體Fibrin400電鏡