Chen Xianqiu, Guo Jian, Yu Dong, Jie Bing, Zhou Ying
Department of Respiratory Medicine, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China.
Department of Pulmonary Function Test, Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China.
Front Pharmacol. 2021 Oct 12;12:754851. doi: 10.3389/fphar.2021.754851. eCollection 2021.
Progressive fibrosing interstitial lung disease (PF-ILD) and idiopathic pulmonary fibrosis (IPF) share similar progression phenotype but with different pathophysiological mechanism. The purpose of this study was to assess clinical characteristics and outcomes of patients with PF-ILD in a single-center cohort. Patients with PF-ILD treated in Shanghai Pulmonary Hospital from Jan. 2013 to Dec. 2014 were retrospectively analyzed. Baseline characteristics and clinical outcomes were collected for survival analysis to identifying clinical predictors of mortality. Among 608 patients with ILD, 132 patients met the diagnostic criteria for PF-ILD. In this single-center cohort, there were 51 (38.6%) cases with connective tissue disease-associated interstitial lung disease (CTD-ILD) and 45 (34.1%) with unclassifiable ILDs. During follow-up, 83 patients (62.9%) either died ( = 79, 59.8%) or underwent lung transplantations ( = 4, 3.0%) with a median duration follow-up time of 53.7 months. Kaplan-Meier survival curves revealed that the 1, 3 and 5-years survival of PF-ILD were 90.9, 58.8 and 48.1%, respectively. In addition, the prognosis of patients with PF-ILD was similar to those with IPF, while it was worse than non-PF-ILD ones. Multivariate Cox regression analysis demonstrated that high-resolution computed tomography (HRCT) scores (HR 1.684, 95% CI 1.017-2.788, = 0.043) and systolic pulmonary artery pressure (SPAP) > 36.5 mmHg (HR 3.619, 95%CI 1.170-11.194, = 0.026) were independent risk factors for the mortality of PF-ILD. Extent of fibrotic changes on HRCT and pulmonary hypertension were predictors of mortality in patients with PF-ILD.
进行性纤维化间质性肺疾病（PF-ILD）和特发性肺纤维化（IPF）具有相似的进展表型，但病理生理机制不同。本研究的目的是评估单中心队列中PF-ILD患者的临床特征和预后。对2013年1月至2014年12月在上海肺科医院接受治疗的PF-ILD患者进行回顾性分析。收集基线特征和临床结局进行生存分析，以确定死亡率的临床预测因素。在608例ILD患者中，132例符合PF-ILD的诊断标准。在这个单中心队列中，有51例（38.6%）结缔组织病相关间质性肺疾病（CTD-ILD）和45例（34.1%）无法分类的ILD。随访期间，83例患者（62.9%）死亡（n = 79，59.8%）或接受肺移植（n = 4，3.0%），中位随访时间为53.7个月。Kaplan-Meier生存曲线显示，PF-ILD患者1年、3年和5年生存率分别为90.9%、58.8%和48.1%。此外，PF-ILD患者的预后与IPF患者相似，但比非PF-ILD患者差。多因素Cox回归分析表明，高分辨率计算机断层扫描（HRCT）评分（HR 1.684，95%CI 1.017 - 2.788，P = 0.043）和收缩期肺动脉压（SPAP）> 36.5 mmHg（HR 3.619，95%CI 1.170 - 11.194，P = 0.026）是PF-ILD患者死亡的独立危险因素。HRCT上纤维化改变的程度和肺动脉高压是PF-ILD患者死亡率的预测因素。
