分析1 316例间质性肺疾病(interstitial lung disease, ILD)患者的疾病谱及临床特点。

收集2015年1月至2021年12月我院收治的1 316例ILD患者的临床资料，分析该人群ILD的疾病谱及临床特点。

纳入病例ILD的疾病谱中，结缔组织病相关间质性肺疾病(connective tissue disease associated interstitial lung disease, CTD-ILD)602例(45.74%)，其中特发性炎性肌病相关间质性肺疾病(idiopathic inflammatory myopathy associated interstitial lung disease, IIM-ILD)174例(13.22%)；特发性间质性肺炎(idiopathic interstitial pneumonia, IIP)536例(40.73%)，其中特发性肺纤维化(idiopathic pulmonary fibrosis, IPF)280例(21.28%)。罕见病339例(25.76%)。60例患者的诊断在随访过程中发生演变，多为IIP患者逐渐出现CTD相关症状及体征，或IPAF患者诊断为CTD-ILD。ILD的影像学分型中，非特异性间质性肺炎( non-specific interstitial pneumonia, NSIP)604例，普通型间质性肺炎(usual interstitial pneumonia, UIP) 414例；CTD-ILD患者胸部影像学主要表现为NSIP、机化性肺炎(organizing pneumonia, OP)、NSIP＋OP。合并症方面，合并恶性肿瘤48例，合并肺动脉高压(pulmonary hypertension, PH)132例。发生急性加重54例(4.10%)。其他并发症还可见感染、气胸、纵隔气肿等。死亡患者27例，其中IPF 7例，IIM-ILD 5例，皮肌炎(DM) 4例，抗合成酶抗体综合征1例，特发性非特异性间质性肺炎4例，自身免疫特征的间质性肺炎4例，类风湿关节炎相关间质性肺疾病(RA-ILD) 3例，隐源性机化性肺炎1例，肺泡性蛋白沉积症1例，多发性肌炎(PM)＋RA-ILD 1例，原发性干燥综合征相关间质性肺疾病1例。

CTD-ILD在ILD人群中所占比例较多。ILD的诊断往往需要动态随访，警惕急性加重、以及并发肺癌、肺动脉高压、感染、气胸、纵隔气肿的发生。

Objective To analyze the disease spectrum and clinical characteristics of 1 316 patients with interstitial lung disease (ILD).

The clinical data of 1316 patients with ILD diagnosed and treated in our hospital from January 2015 to December 2021 were collected, and the disease spectrum and clinical characteristics of ILD in this population were analyzed.

In the disease spectrum of ILD cases, 602 cases (45.74%) were connective tissue disease associated interstitial lung disease (CTD-ILD), including 174 cases (13.22%) of idiopathic inflammatory myopathy associated interstitial lung disease (IIM-ILD). 339 cases (25.76%) were rare diseases. The diagnosis of 60 patients evolved during the follow-up, most of them were IIP patients who gradually developed CTD related symptoms and signs, or IPAF patients diagnosed with CTD-ILD. Among the imaging subtypes of ILD, 604 cases were non-specific interstitial pneumonia (NSIP) and 414 cases were common interstitial pneumonia (UIP); Chest imaging of CTD-ILD patients mainly showed NSIP, organized pneumonia (OP), NSIP+ OP. In terms of comorbidities, 48 cases were complicated with malignant tumors and 132 cases with pulmonary hypertension (PH). Acute exacerbation occurred in 54 cases (4.10%). Other complications include infection, pneumothorax, mediastinal emphysema, etc.

CTD-ILD accounts for a large proportion in ILD population. The diagnosis of ILD often requires dynamic follow-up, and the occurrence of acute exacerbation, lung cancer, pulmonary hypertension, infection, pneumothorax, and mediastinal emphysema should be vigilant.