ANCA相关性血管炎肾损害病理表现.ppt

ANCA相关性血管炎肾损害病理表现,P-ANCA Perinuclear PatternAnti-myeloperoxidaseMPO-ANCA,C-ANCA Cytoplasmic PatternAnti-Proteinase 3PR3-ANCA,Anti-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 Nomenclature,光镜肾小球节段性毛细血管袢纤维素样坏死新月体性肾炎，多新旧不等肾小动脉10%-50%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肾间质-小管炎症，偶见肉芽肿样病变偶见髓质小管周围炎免疫病理和电镜微量或阴性,ANCA相关小血管炎肾损害病理表现,毛细血管袢纤维素样坏死,新月体形成,细胞性新月体,纤维细胞性新月体,小动脉纤维素样坏死,小叶间动脉纤维素样坏死肾间质大量炎症细胞浸润,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肾间质大量炎症细胞浸润,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 血管腔内膜和肌层均纤维化,小管间质病变-炎细胞浸润,小管间质病变-炎细胞浸润,小管间质病变-炎细胞浸润,小管间质病变-坏死性肉芽肿形成,免疫荧光-寡免疫性,IgG400,免疫荧光,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,Fibrin400,电镜,毛细血管袢基底膜断裂,电镜,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。,