摘要目的:探究血清抗中性粒细胞胞质抗体（ANCA）阳性的间质性肺疾病患者的临床特点及预后。方法:回顾性纳入2006年3月至2016年3月就诊于北京协和医院的间质性肺疾病患者274例，其中男81例，女193例，年龄（53±11）岁，根据诊断分为血清ANCA阳性肺间质病（ANCA-ILD）、结缔组织病相关性肺间质病和自身免疫特征的间质性肺炎（CTD-ILD/IPAF）及特发性间质性肺炎（IIP）组，分析3组患者在临床表现、血清学、肺功能、影像学、生存及复发方面的差异。结果:274例患者中ANCA阳性38例（38/274，14%），发病年龄（59±10）岁，随访时间（52±31）个月，死亡7例（7/38，18%）。ANCA阳性患者发病年龄高于CTD-ILD/IPAF[（52±10）岁]和IIP[（53±11）岁， H=19.29， P<0.001]，血红蛋白[（129±21）mg/L]低于CTD-ILD/IPAF[（138±15）mg/L]和IIP[（140±19）mg/L， H=8.17， P=0.017]，ESR[（46±35）mm/1 h]高于CTD-ILD/IPAF[（26±24）mm/1 h]和IIP[（19±22）mm/1 h， H=19.73， P<0.001]，治疗后肺功能FVC改善率（31%）低于CTD-ILD/IPAF（59%）和IIP（39%， χ2=11.74， P=0.003），胸部CT病变吸收率（61%）低于CTD-ILD/IPAF（82%）和IIP（67%， χ2=9.23， P=0.010），病死率（18%）高于CTD-ILD/IPAF（6%）和IIP（12%, χ2=7.16， P=0.028）。 结论:ANCA阳性患者与其他类型肺间质病患者在临床特征方面存在差异，治疗效果不佳，预后较差。

abstractsObjective:To investigate the clinical features and prognosis of interstitial lung disease patients with positive anti-neutrophil cytoplasmic antibody.Methods:The patients with interstitial lung disease who visited Peking Union Medical College Hospital from March 2006 to March 2016 were divided into three groups: interstitial lung disease with ANCA-positive(ANCA-ILD), connective tissue disease associated interstitial lung disease and interstitial pneumonia with autoimmune features (CTD-ILD/IPAF) and idiopathic interstitial pneumonia (IIP). The three groups were analyzed in terms of clinical manifestations, serology, lung function, imaging, survival and recurrence.Results:Two hundred and seventy four patients were enrolled and 38 (14%) were ANCA-positive of whom 16 were male and 22 were female. The age of 38 ANCA-positive patients was (59±10) years and the follow-up time was (52±31) months. Seven among the 38 ANCA-positive patients died and the death rate is 18.42%. The ANCA-positive patients with interstitial lung disease have higher onset age (ANCA-ILD:59±10,CTD-ILD/IPAF:52±10,IIP:53±11, H=19.29, P<0.001), lower hemoglobin (ANCA-ILD: 129±21, CTD-ILD/IPAF: 138±15, IIP: 140±19, H=8.17, P=0.017), higher erythrocyte sedimentation rate (ANCA-ILD:45±35, CTD-ILD/IPAF:26±24,IIP:19±22, H=19.73, P<0.001), lower lung function improvement rate after treatment (ANCA-ILD:31%,CTD-ILD/IPAF:59%,IIP: 39%, χ2=11.74, P=0.003), lower absorption rate of CT lesion (ANCA-ILD:61%,CTD-ILD/IPAF:82%,IIP:67%, χ2=9.23, P=0.010) and higher death rate(ANCA-ILD:18%,CTD-ILD/IPAF:6%,IIP:12%, χ2=7.16, P=0.028). Conclusions:There are significant differences in clinical characteristics between ANCA-positive patients and other types of pulmonary interstitial disease. And both the treatment effect and the prognosis is poor for the ANCA-positive patients.