表现为肺纤维化的抗中性粒细胞胞浆抗体相关性小血管炎临床分析

目的 探讨肺部病变表现为纤雏化的抗中性粒细胞胞浆抗体（ANCA）相关性小血管炎的临床特征。方法 回顾性分析10例以肺纤维化为主要肺部病变的ANCA相关性小血管炎患者临床资料。结果 以肺纤维化为主要表现的患者共10例，占同期住院ANCA相关性小血管炎患者的25．0％。胸部CT表现为程度不一的肺纤维化，其中5例类似寻常型间质性肺炎。所有患者均为ANCA阳性，9例为抗髓过氧化物抗体阳性．1例为抗蛋白酶3抗体阳性。10例患者中2例仅有肺纤维化的表现；8例合并肾脏病变，其中6例呼吸道症状先于肾损害出现，2例同时出现。结论 肺纤维化可作为ANCA相关性小血管炎的早期表现，原因不明的肺纤维化患者应常规进行ANCA检查。

Clinical analysis of antineutrophil cytoplasmic autoantibody-associated vasculitis manifested as pulmonary fibrosis

Objective To explore the clinical features of antineutrophil cytoplasmic autoantibody(ANCA)-associated vasculitis manifested as pulmonary fibrosis.Methods Clinical data of 10 cases of ANCA-associated vasculitis manifested as pulmonary fibrosis were retrospectively analyzed.Results There were 10 cases of ANCA-associated vasculitis manifested as pulmonary fibrosis,which accounted for 25.0% of all the cases in the same period.The chest CT scan showed pulmonary fibrosis of different extent,among them 5 cases were similar as usual interstitial pneumonia (UIP).All of the patients were ANCA positive,90% were anti-MPO antibody positive and 10% were anti-PR3 antibody positive.2/10 cases were manifested only as pulmonary fibrosis.8/10 cases with pulmonary fibrosis were complicated with renal diseases,in whom respiratory symptoms occurred in 75% cases prior to kidney damage,and 2/8 cases developed respiratory symptoms and renal damage at the same time.Conclusion Pulmonary fibrosis might be the early manifestation of ANCA-associated vasculitis.ANCA should be examined in patients with pulmonary fibrosis of unknown causes.