ANCA相关性血管炎肾损害病理表现.ppt

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

2、a 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 Associate

4、d with Probable Etiologies (e.g. HBV, HCV, drug, cancer, etc.),2011 Chapel Hill Consensus Conference Vasculitis Nomenclature,a,4,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,a,5,毛细血管袢纤维素样坏死,a,6,新月体形成,细胞性新月体,纤维细胞性新月体,a,7,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,a,8,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,a,9,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,a,10,小管间质病变-炎细胞浸润,a,11,小管间质病变-炎细胞浸润,a,12,小管间质病变-炎细胞浸润,a,13,小管间质病变-坏死性肉芽肿形成,a,14,免疫荧光-寡免疫性,IgG400,a,15,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