【摘要】 目的　分析ANCA相关性小血管性肺炎的病理基础及影像学特点 ,提示其诊断与鉴别诊断要点及预后。方法　选择 2 0 0 1年 10月～ 2 0 0 4年 3月在我院住院经实验室测检ANCA均为阳性患者 7例 ,有多次胸部X线照片 ,4例有CT扫描 ,2例行肾穿刺活检。结果　 7例患者均确诊为ANCA相关性小血管炎 ,表现为多系统多脏器损害。经治疗 2～ 6周后6例肺部病变完全吸收消失 ,1例因多器官衰竭抢救无效死亡。结论　抗中性粒细胞胞浆抗体 (ANCA)相关性小血管炎属少见的自身免疫性疾病 ,肺脏是最常累及的脏器之一 ,其肺部影像表现并无特异性改变 ,对有肺脏受累的多系统损害的病人 ,特别是有咯血者 ,要想到本病的可能 ,及时确诊 ,临床给予甲基强的松龙和环磷酰胺双冲击治疗 ,病情在短期内即有显著好转 ,预后较好 ,其肺部的反应明显好于其他系统的损害更多还原

【Abstract】 Objective To reveal the key point of diagnosis and distinctions as well as the prognosis by analyzing the characteristics of pulmonary imaging manifestation and pathologic foundation in ANCA associated vasculitis. Methods 7 cases with positive ANCA laboratory results from Oct 2001-Mar 2004 had been taken plain chest radiography for several times.4 cases had CT scan, 2 cases had kidney biopsy. Results 7 cases with ANCA associated vasculitis showed multi-organ damages.After treatment for 2-6 weeks, the lung damaging manifestation disappeared in 6 cases, and one died with multi-organ failure. Conclusion ANCA associated vasculitis is a rare autoimmune disease. The lung is one of the organs involved. There is no specific manifestations in imaging. ANCA associated vasculitis should be considered when patients have multi-system damages besides lung, especially for thosewith hemoptysis.Diagnosis should be made in time. The symptoms and manifestation of lungs can be controlled quickly after carrying out double pulse therapy of methylprednisolone and cyclophosphmide.The prognosis is good.The lung damage to double pulse therapy is better than other systems damages.更多还原