ANCA相关性血管炎肾损害病理表现.pptx

ANCA相关性血管炎肾损害病理表现ANCA相关性血管炎肾损害病理表现第1页P-ANCA Perinuclear PatternAnti-myeloperoxidaseMPO-ANCAC-ANCA Cytoplasmic PatternAnti-Proteinase 3PR3-ANCAAnti-Neutrophil Cytoplasmic Autoantibodies(ANCA)ANCA相关性血管炎肾损害病理表现第2页 Large Vessel VasculitisGiant Cell ArteritisTakayasu ArteritisMedium Vessel VasculitisPolyarteritis NodosaKawasaki DiseaseSmall Vessel 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 Urticarial 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,HCV,drug,cancer,etc.)Chapel Hill Consensus Conference Vasculitis NomenclatureANCA相关性血管炎肾损害病理表现第3页光镜光镜肾小球肾小球节段性毛细血管袢纤维素样坏死节段性毛细血管袢纤维素样坏死新月体性肾炎，多新旧不等新月体性肾炎，多新旧不等肾小动脉肾小动脉10%-50%10%-50%可有肾小球外小动脉纤维素样坏死，部分可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有中等动脉受累肾间质肾间质-小管小管炎症，偶见肉芽肿样病变炎症，偶见肉芽肿样病变偶见髓质小管周围炎偶见髓质小管周围炎免疫病理和电镜免疫病理和电镜微量或阴性微量或阴性ANCA相关小血管炎肾损害病理表现ANCA相关性血管炎肾损害病理表现第4页毛细血管袢纤维素样坏死ANCA相关性血管炎肾损害病理表现第5页新月体形成细胞性新月体细胞性新月体纤维细胞性新月体纤维细胞性新月体ANCA相关性血管炎肾损害病理表现第6页小动脉纤维素样坏死小叶间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肾间质大量炎症细胞浸润ANCA相关性血管炎肾损害病理表现第7页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肾间质大量炎症细胞浸润ANCA相关性血管炎肾损害病理表现第8页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血管腔内膜和肌层均纤维化ANCA相关性血管炎肾损害病理表现第9页小管间质病变-炎细胞浸润ANCA相关性血管炎肾损害病理表现第10页小管间质病变-炎细胞浸润ANCA相关性血管炎肾损害病理表现第11页小管间质病变-炎细胞浸润ANCA相关性血管炎肾损害病理表现第12页小管间质病变-坏死性肉芽肿形成ANCA相关性血管炎肾损害病理表现第13页免疫荧光-寡免疫性IgG400ANCA相关性血管炎肾损害病理表现第14页免疫荧光左：纤维素样坏死左：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体右：从一个纤维素样坏死病灶蔓延至相邻小新月体Fibrin400ANCA相关性血管炎肾损害病理表现第15页电镜毛细血管袢基底膜断裂毛细血管袢基底膜断裂ANCA相关性血管炎肾损害病理表现第16页电镜血管袢基底膜中止，纤维蛋白从破裂毛细血管血管袢基底膜中止，纤维蛋白从破裂毛细血管袢漏入右边鲍曼囊腔。袢漏入右边鲍曼囊腔。ANCA相关性血管炎肾损害病理表现第17页