单击此处编辑母版标题样式,单击此处编辑母版文本样式,第二级,第三级,第四级,第五级,*,精选课件,*,ANCA,相关性血管炎肾损害,病理表现,1,精选课件,ANCA相关性血管炎肾损害病理表现1精选课件,P-ANCA,Perinuclear Pattern,Anti-myeloperoxidase,MPO-ANCA,C-ANCA,Cytoplasmic Pattern,Anti-Proteinase 3,PR3-ANCA,Anti-Neutrophil Cytoplasmic Autoantibodies(ANCA),2,精选课件,P-ANCA Perinuclear PatternC-AN,Large Vessel Vasculitis,Giant Cell Arteritis,Takayasu Arteritis,Medium Vessel Vasculitis,Polyarteritis Nodosa,Kawasaki Disease,Small Vessel Vasculitis,ANCA-Associated Vasculitis,（,AASV,）,Microscopic Polyangiitis,Granulomatosis with Polyangiitis(Wegeners),Eosinophilic Granulomatosis with Polyangiitis(Churg-Strauss),Immune Complex Vasculitis,Anti-GBM Disease,IgA Vasculitis(Henoch-Schnlein),Cryoglobulinemic Vasculitis,Hypocomplementemic 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,3,精选课件,Large Vessel Vasculitis2011 C,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,4,精选课件,光镜ANCA相关小血管炎肾损害病理表现4精选课件,毛细血管袢纤维素样坏死,5,精选课件,毛细血管袢纤维素样坏死5精选课件,新月体形成,细胞性新月体,纤维细胞性新月体,6,精选课件,新月体形成细胞性新月体纤维细胞性新月体6精选课件,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,7,精选课件,小动脉纤维素样坏死小叶间动脉纤维素样坏死7精选课件,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,8,精选课件,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,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,9,精选课件,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,小管间质病变,-,炎细胞浸润,10,精选课件,小管间质病变-炎细胞浸润10精选课件,小管间质病变,-,炎细胞浸润,11,精选课件,小管间质病变-炎细胞浸润11精选课件,小管间质病变,-,炎细胞浸润,12,精选课件,小管间质病变-炎细胞浸润12精选课件,小管间质病变,-,坏死性肉芽肿形成,13,精选课件,小管间质病变-坏死性肉芽肿形成13精选课件,免疫荧光,-,寡免疫性,IgG400,14,精选课件,免疫荧光-寡免疫性IgG40014精选课件,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,Fibrin400,15,精选课件,免疫荧光左：纤维素样坏死Fibrin40015精选课件,电镜,毛细血管袢基底膜断裂,16,精选课件,电镜毛细血管袢基底膜断裂16精选课件,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。,17,精选课件,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,