TY - JOUR T1 - Progressive fibrosing interstitial lung disease: a clinical cohort (the PROGRESS® study) JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.02718-2020 SP - 2002718 AU - Mouhamad Nasser AU - Sophie Larrieu AU - Salim Si-Mohamed AU - Kaïs Ahmad AU - Loic Boussel AU - Marie Brevet AU - Lara Chalabreysse AU - Céline Fabre AU - Sébastien Marque AU - Didier Revel AU - Françoise Thivolet-Bejui AU - Julie Traclet AU - Sabrina Zeghmar AU - Delphine Maucort-Boulch AU - Vincent Cottin Y1 - 2020/01/01 UR - http://erj.ersjournals.com/content/early/2020/08/28/13993003.02718-2020.abstract N2 - 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 retrospectively examined for predefined 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 women. Baseline mean forced vital capacity (FVC) was 74±22% of 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.FootnotesThis manuscript has recently been accepted for publication in the European Respiratory Journal. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJ online. Please open or download the PDF to view this article.Conflict of interest: Dr Nasser received sponsorship for conference from Boerhinger Ingelhium and Hoffmann-La Roche. Dr Nasser received consultation fees from Boerhinger Ingelhium.Conflict of interest: Dr. Larrieu has nothing to disclose.Conflict of interest: Dr. Si-mohamed has nothing to disclose.Conflict of interest: Dr. Ahmad reports other from Roche, other from Boehringer Ingelheim, outside the submitted work.Conflict of interest: Dr. BOUSSEL has nothing to disclose.Conflict of interest: Dr. BREVET has nothing to disclose.Conflict of interest: Dr. Chalabreysse has nothing to disclose.Conflict of interest: Dr. Fabre has nothing to disclose.Conflict of interest: Dr. Marque has nothing to disclose.Conflict of interest: Dr. Revel has nothing to disclose.Conflict of interest: Dr. THIVOLET-BEJUI has nothing to disclose.Conflict of interest: Dr. Traclet reports other from Roche, other from Boehringer Ingelheim, outside the submitted work.Conflict of interest: Dr. Zeghmar has nothing to disclose.Conflict of interest: Dr. Maucort-Boulch has nothing to disclose.Conflict of interest: Dr. Cottin reports personal fees and non-financial support from Actelion, grants, personal fees and non-financial support from Boehringer Ingelheim, personal fees from Bayer/MSD, personal fees from Novartis, grants, personal fees and nonfinancial support from Roche, personal fees from Sanofi, personal fees from Promedior, personal fees from Celgene, personal fees from Galapagos, personal fees from Galecto, outside the submitted work. ER -