血清抗中性粒细胞胞质抗体阳性肺间质病患者临床及预后分析

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%,χ^2=11.74,P=0.003),胸部CT病变吸收率(61%)低于CTD-ILD/IPAF(82%)和IIP(67%,χ^2=9.23,P=0.010),病死率(18%)高于CTD-ILD/IPAF(6%)和IIP(12%,χ^2=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。

The clinical features and prognosis of interstitial lung disease patients with positive anti-neutrophil cytoplasmic antibody

Objective 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%,χ^2=11.74,P=0.003),lower absorption rate of CT lesion(ANCA-ILD:61%,CTD-ILD/IPAF:82%,IIP:67%,χ^2=9.23,P=0.010)and higher death rate(ANCA-ILD:18%,CTD-ILD/IPAF:6%,IIP:12%,χ^2=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.