内容·我国ANCA相关小血管炎的特点·临床表现·诊断·治疗原发性系统性小面管·韦格纳肉芽肿病(WG)●1变应性肉芽肿性血管炎(CSS)·显微镜下型多血管炎(MPA)●过敏性紫癜·原发性冷球蛋白血症性血管炎·皮肤白细胞碎裂性血管炎·最近的变化。WG---GPA:Granulomatosis with polyangiitis芽肿性血管炎)。CSS--EGPA:Eosinophilic GPA(嗜酸性肉芽肿血管炎)Anti-GBM disease(抗肾小球基底膜疾病)Jennette et al.Arthritis Rheum 1994:37:187-192ANCA相关小血管炎的肾脏病理分型基于肾小球病变(5o%)50%globallyYESSclerotic·硬化sclerotic glomeruliclass·正常NO。细胞新月体250%normalYESFocalglomeruliclass100FocalNO801Crescentic250%cellularYESCrescentic60crescentsclassMixed40-Sclerotic20NOMixedclassFocalCreecentio-Mixed-Sclerotiocensoredcensoredceneoredcensored268102Follow up in years to renal failureBajema,IM,et al.J Am Soc Nephrol.2010;21:1628-36.