Variable Vessel Vasculitis (Cogan’s, Behcet’s, 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 Etio.logies (e.g. HBV, HCV, drug, cancer, etc.)
ANCA相关小血管炎肾损害病理表现
• 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
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毛细血管袢纤维素样坏死
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新月体形成
细胞性新月体
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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
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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
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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
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小管间质病变-炎细胞浸润
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小管间质病变-炎细胞浸润
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小管间质病变-炎细胞浸润
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小管间质病变-坏死性肉芽肿形成
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免疫荧光-寡免疫坏死
Fibrin×40 0
右：从一个纤维素样坏死. 病灶蔓延至相邻小新月体
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。
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2011 Chapel Hill Consensus Conference Vasculitis Nomenclature
Large Vessel Vasculitis Giant Cell Arteritis Takayasu Arteritis
Medium Vessel Vasculitis Polyarteritis Nodosa Kawasaki Disease
ANCA相关性血管炎肾损 害
病理表现
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Anti-Neutrophil Cytoplasmic Autoantibodies (ANCA)
C-ANCA Cytoplasmic Pattern Anti-Proteinase 3 PR3-ANCA
P-ANCA Perinuclear Pattern Anti-myeloperoxidase MPO-ANCA
Small Vessel Vasculitis ANCA-Associated Vasculitis（AASV） Microscopic Polyangiitis Granulomatosis with Polyangiitis (Wegener’s) Eosinophilic Granulomatosis with Polyangiitis (Churg-Strauss) Immune Complex Vasculitis Anti-GBM Disease IgA Vasculitis (Henoch-Schönlein) Cryoglobulinemic Vasculitis Hypocomplementemic Urticarial Vasculitis (Anti-C1q Vasculitis)