1、ANCA相关性血管炎肾损害病理表现1.P-ANCA Perinuclear PatternAnti-myeloperoxidaseMPO-ANCAC-ANCA Cytoplasmic PatternAnti-Proteinase 3PR3-ANCAAnti-Neutrophil Cytoplasmic Autoantibodies (ANCA)2 Large Vessel VasculitisGiant Cell ArteritisTakayasu ArteritisMedium Vessel VasculitisPolyarteritis NodosaKawasaki DiseaseSmal
2、l 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 Ur
3、ticarial 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
4、, HCV, drug, cancer, etc.) 2011 Chapel Hill Consensus Conference Vasculitis Nomenclature3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
5、微量或阴性ANCA相关小血管炎肾损害病理表现4毛细血管袢纤维素样坏死5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6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7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8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9小管间质病变-炎细胞浸润10小管间质病变-炎细胞浸润11小管间质病变-炎细胞浸润12小管间质病变-坏死性肉芽肿形成13免疫荧光-寡免疫性IgG40014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Fibrin40015电镜毛细血管袢基底膜断裂毛细血管袢基底膜断裂16电镜血管袢基底膜中断，纤维蛋白从破裂的毛细血血管袢基底膜中断，纤维蛋白从破裂的毛细血管袢漏入右边鲍曼囊腔。管袢漏入右边鲍曼囊腔。17
