Gao C, Sun X, Huang J, Peng M, Sun X F, Zhang T, Shi J H
Department of Respiratory Medicine, Peking Union Medical College Hospital, Peking Union Medical College and Chinese Academy of Medical Sciences, Beijing 100730, China.
Zhonghua Jie He He Hu Xi Za Zhi. 2020 Apr 12;43(4):362-368. doi: 10.3760/cma.j.cn112147-20191205-00813.
To investigate the clinical features and prognosis of interstitial lung disease patients with positive anti-neutrophil cytoplasmic antibody. 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. 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,=19.29, 0.001), lower hemoglobin (ANCA-ILD: 129±21, CTD-ILD/IPAF: 138±15, IIP: 140±19, =8.17, 0.017), higher erythrocyte sedimentation rate (ANCA-ILD:45±35, CTD-ILD/IPAF:26±24,IIP:19±22,=19.73, 0.001), lower lung function improvement rate after treatment (ANCA-ILD:31%,CTD-ILD/IPAF:59%,IIP: 39%,(2)=11.74,0.003), lower absorption rate of CT lesion (ANCA-ILD:61%,CTD-ILD/IPAF:82%,IIP:67%, (2)=9.23,0.010) and higher death rate(ANCA-ILD:18%,CTD-ILD/IPAF:6%,IIP:12%, (2)=7.16,0.028). 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.
探讨抗中性粒细胞胞浆抗体阳性的间质性肺疾病患者的临床特征及预后。将2006年3月至2016年3月在北京协和医院就诊的间质性肺疾病患者分为三组：抗中性粒细胞胞浆抗体阳性的间质性肺疾病（ANCA-ILD）、结缔组织病相关间质性肺疾病和具有自身免疫特征的间质性肺炎（CTD-ILD/IPAF）以及特发性间质性肺炎（IIP）。对三组患者的临床表现、血清学、肺功能、影像学、生存及复发情况进行分析。共纳入274例患者，其中38例（14%）抗中性粒细胞胞浆抗体阳性，男性16例，女性22例。38例抗中性粒细胞胞浆抗体阳性患者年龄为（59±10）岁，随访时间为（52±31）个月。38例抗中性粒细胞胞浆抗体阳性患者中7例死亡，死亡率为18.42%。抗中性粒细胞胞浆抗体阳性的间质性肺疾病患者发病年龄较高（ANCA-ILD：59±10，CTD-ILD/IPAF：52±10，IIP：53±11，F=19.29，P=0.001），血红蛋白较低（ANCA-ILD：129±21，CTD-ILD/IPAF：138±15，IIP：140±19，F=8.17，P=0.017），红细胞沉降率较高（ANCA-ILD：45±35，CTD-ILD/IPAF：26±24，IIP：19±22，F=19.73，P=0.001），治疗后肺功能改善率较低（ANCA-ILD：31%，CTD-ILD/IPAF：59%，IIP：39%，χ2=11.74，P=0.003），CT病变吸收率较低（ANCA-ILD：61%，CTD-ILD/IPAF：82%，IIP：67%，χ2=9.23，P=0.010），死亡率较高（ANCA-ILD：18%，CTD-ILD/IPAF：6%，IIP：12%，χ2=7.16，P=0.028）。抗中性粒细胞胞浆抗体阳性患者与其他类型的肺间质疾病在临床特征上存在显著差异。抗中性粒细胞胞浆抗体阳性患者的治疗效果及预后均较差。
