ANCA相关性血管炎肾损害病理表现.pptx

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,2016/12/30,#,ANCA,有关性血管炎肾损害,病理体现,第1页,P-ANCA,Perinuclear Pattern,Anti-myeloperoxidase,MPO-ANCA,C-ANCA,Cytoplasmic Pattern,Anti-Proteinase 3,PR3-ANCA,Anti-Neutrophil Cytoplasmic Autoantibodies(ANCA),第2页,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.),2023 Chapel Hill Consensus Conference Vasculitis Nomenclature,第3页,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，部分可有中档动脉受累,肾间质,-,小管,炎症，偶见肉芽肿样病变,偶见髓质小管周边炎,免疫病理和电镜,微量或阴性,ANCA有关,小,血管炎肾损害病理体现,第4页,毛细血管袢纤维素样坏死,第5页,新月体形成,细胞性新月体,纤维细胞性新月体,第6页,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,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肾间质大量炎症细胞浸润,第8页,小动脉纤维素样坏死,慢性化体现：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,第9页,小管间质病变,-,炎细胞浸润,第10页,小管间质病变,-,炎细胞浸润,第11页,小管间质病变,-,炎细胞浸润,第12页,小管间质病变,-,坏死性肉芽肿形成,第13页,免疫荧光,-,寡免疫性,IgG,400,第14页,免疫荧光,左：纤维素样坏死,右：从一种纤维素样坏死病灶蔓延至相邻小新月体,Fibrin,400,第15页,电镜,毛细血管袢基底膜断裂,第16页,电镜,血管袢基底膜中断，纤维蛋白从破裂旳毛细血管袢漏入右边鲍曼囊腔。