ANCA相关性血管炎肾损害病理表现.ppt

ANCA相关性血管炎肾损相关性血管炎肾损害害病理表现病理表现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 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.)2011 Chapel Hill Consensus Conference Vasculitis Nomenclaturel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l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相关相关小小血管炎肾损害病理表现血管炎肾损害病理表现毛细血管袢纤维素样坏死毛细血管袢纤维素样坏死新月体形成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小动脉纤维素样坏死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小动脉纤维素样坏死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小动脉纤维素样坏死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小管间质病变小管间质病变-炎细胞浸润炎细胞浸润小管间质病变小管间质病变-炎细胞浸润炎细胞浸润小管间质病变小管间质病变-炎细胞浸润炎细胞浸润小管间质病变小管间质病变-坏死性肉芽肿形成坏死性肉芽肿形成免疫荧光免疫荧光-寡免疫性寡免疫性IgG400免疫荧光免疫荧光左：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Fibrin400电镜电镜毛细血管袢基底膜断裂毛细血管袢基底膜断裂电镜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。