具有自身免疫特征的间质性肺炎的临床特点

Clinical Characteristics of Interstitial Pneumonia with Autoimmune Features

目的探讨具有自身免疫特征的间质性肺炎(interstitial pneumonia with autoimmune features,IPAF)患者的临床特征,比较IPAF与非IPAF-IIP(特发性间质性肺炎)、IPF(特发性肺纤维化)的临床差异。方法纳入2014年1月至2019年12月在昆明医科大学第二附属医院呼吸内科、风湿免疫科连续性诊断为IIP(n=130)的患者,其中IPAF66例,不能诊断为IPAF的IIP患者(非IPAF-IIP)64例,在非IPAF-IIP中找出符合IPF诊断的患者为IPF组42例。收集患者的一般情况、临床症状体征及辅助检查等临床资料,对各组不同指标数据进行统计分析,明确其特点及差异性。结果在130例IIP患者中,有66例(50.8%)被重新分类为IPAF,在64例(49.2%)非IPAF-IIP中有42例(32.3%)IPF。IPAF主要以形态学及血清学领域达到诊断标准为主。三个领域中分别以炎性关节炎或多关节晨僵≥60 min、ANA、NSIP为主要表现;相比于非IPAF-IIP及IPF,IPAF以不吸烟女性患者为主,激素使用率更高,并且具有较高的DLCO、FVC和较低的肺动脉压,三组患者临床表现相似,鉴别困难,女性、胸腔多部位受累及临床体征的发现可能有助于诊断,三组之间生存率差异无统计学意义(P> 0.05),但IPAF中位生存时间大于非IPAF-IIP及IPF。结论 (1)IPAF主要以形态学及血清学领域达到诊断标准为主;(2)HRCT主要以NSIP为主,血清学领域主要以ANA阳性为主;(3)IPAF激素使用率明显高于非IPAF-IIP及IPF。

Objective To explore the clinical features of interstitial pneumonia(IPAF)patients with autoimmune characteristics,and to compare the clinical difference between IPAF and non-IPAF-IIP(idiopathic interstitial pneumonia)and IPF(idiopathic pulmonary fibrosis).Methods Patients(n=130)diagnosed with IIP consecutively in Department of Respiratory Medicine and Department of Rheumatology and Immunology of the Second Affiliated Hospital of Kunming Medical University from January 2014 to December 2019 were included,including 66 cases of IPAF,64 cases of IIP that could not be diagnosed as IPAF(non-IPAF-IIP);and in the non-IPAF-IIP group,42 patients in the IPF group were found to meet the IPF diagnosis.The demographics,clinical symptoms and signs,lab results and other clinical data of the patients were collected,and statistical analysis was conducted on the data of different indicators in each group to differentiate their characteristics.Results Among 130 IIP patients,66(50.8%)were reclassified as IPAF,and 42(32.3%)were reclassified as IPF among non-IPAFIIP(64(49.2%)).IPAF diagnosis is mainly based on criteria in morphology and serology.Inflammatory arthritis or multi-joint morning stiffness≥60 min,ANA and NSIP were the main manifestations in the three fields.Compared with non-IPAF-IIP and IPF,IPAF is more commonly seen in non-smoking female patients,with higher hormone using rate and higher DLCO,FVC and lower pulmonary artery pressure.The clinical manifestations of the three groups are similar,making it difficult to differentiate.Female,multiple chest lesions and clinical signs may be helpful for differential diagnosis.There is no significant difference in survival rate among the three groups(P>0.05),but the median survival time of IPAF is higher than non-IPAF-IIP and IPF.Conclusion(1)IPAF diagnosis is mainly based on criteria in morphology and serology;(2)NSIP is the main type of HRCT,and ANA is the main serological type;(3)The hormone using rate of IPAF was significantly higher than non-IPAF-IIP and IPF.