电子T1进步fi RT老杂志文章brosing interstitial lung disease: a clinical cohort (the PROGRESS study) JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP 2002718 DO 10.1183/13993003.02718-2020 VO 57 IS 2 A1 Nasser, Mouhamad A1 Larrieu, Sophie A1 Si-Mohamed, Salim A1 Ahmad, Kaïs A1 Boussel, Loic A1 Brevet, Marie A1 Chalabreysse, Lara A1 Fabre, Céline A1 Marque, Sébastien A1 Revel, Didier A1 Thivolet-Bejui, Françoise A1 Traclet, Julie A1 Zeghmar, Sabrina A1 Maucort-Boulch, Delphine A1 Cottin, Vincent YR 2021 UL //www.qdcxjkg.com/content/57/2/2002718.abstract AB In patients with chronic fibrosing interstitial lung disease (ILD), a progressive fibrosing phenotype (PF-ILD) may develop, but information on the frequency and characteristics of this population outside clinical trials is lacking.We assessed the characteristics and outcomes of patients with PF-ILD other than idiopathic pulmonary fibrosis (IPF) in a real-world, single-centre clinical cohort. The files of all consecutive adult patients with fibrosing ILD (2010–2017) were examined retrospectively for pre-defined criteria of ≥10% fibrosis on high-resolution computed tomography and progressive disease during overlapping windows of 2 years. Baseline was defined as the date disease progression was identified. Patients receiving nintedanib or pirfenidone were censored from survival and progression analyses.In total, 1395 patients were screened; 617 had ILD other than IPF or combined pulmonary fibrosis and emphysema, and 168 had progressive fibrosing phenotypes. In 165 evaluable patients, median age was 61 years; 57% were female. Baseline mean forced vital capacity (FVC) was 74±22% predicted. Median duration of follow-up was 46.2 months. Annualised FVC decline during the first year was estimated at 136±328 mL using a linear mixed model. Overall survival was 83% at 3 years and 72% at 5 years. Using multivariate Cox regression analysis, mortality was significantly associated with relative FVC decline ≥10% in the previous 24 months (p<0.05), age ≥50 years (p<0.01) and diagnosis subgroup (p<0.01).In this cohort of patients with PF-ILD not receiving antifibrotic therapy, the disease followed a course characterised by continued decline in lung function, which predicted mortality.In a real-world clinical cohort (PROGRESS), progressive fibrosing interstitial lung disease was characterised by continued lung function decline. Lung function decline, age and underlying diagnosis subgroup predicted mortality. https://bit.ly/2EB3OpF