ANCA相关性血管炎肾损害病理表现【业界研究】

1、ANCA相关性血管炎肾损害病理表现,1,专业倾力,P-ANCA Perinuclear Pattern Anti-myeloperoxidase MPO-ANCA,C-ANCA Cytoplasmic Pattern Anti-Proteinase 3 PR3-ANCA,Anti-Neutrophil Cytoplasmic Autoantibodies (ANCA),2,专业倾力,Large Vessel Vasculitis Giant Cell Arteritis Takayasu Arteritis Medium Vessel Vasculitis Polyarteritis Nodo

2、sa 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

3、 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 Associat

4、ed with Probable Etiologies (e.g. HBV, HCV, drug, cancer, etc.),2011 Chapel Hill Consensus Conference Vasculitis Nomenclature,3,专业倾力,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,专业倾力,毛细血管袢纤维素样坏死,5,专业倾力,新月体形成,细

5、胞性新月体,纤维细胞性新月体,6,专业倾力,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,专业倾力,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,专业倾力,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,专业倾力,小管间质病变-炎细胞浸润,10,专业倾力,小管间质病变-炎细胞浸润,11,专业倾力,小管间质病变-炎细胞浸润,12,专业倾力,小管间质病变-坏死性肉芽肿形成,13,专业倾力,免疫荧光-寡免疫性,IgG400,14,专业倾力,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,Fib