1. Progressive fibrosing interstitial lung disease: a clinical cohort (the PROGRESS study)
- Author
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Sébastien Marque, Marie Brevet, Lara Chalabreysse, Didier Revel, Françoise Thivolet-Béjui, Vincent Cottin, Julie Traclet, Delphine Maucort-Boulch, Céline Fabre, Salim Si-Mohamed, K. Ahmad, Loic Boussel, Sabrina Zeghmar, Sophie Larrieu, Mouhamad Nasser, Infections Virales et Pathologie Comparée - UMR 754 (IVPC), École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), and Boehringer Ingelheim
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Adult ,Pulmonary and Respiratory Medicine ,medicine.medical_specialty ,Vital Capacity ,Population ,Interstitial Lung Disease ,[SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract ,03 medical and health sciences ,Idiopathic pulmonary fibrosis ,FEV1/FVC ratio ,0302 clinical medicine ,Internal medicine ,medicine ,Humans ,030212 general & internal medicine ,education ,Retrospective Studies ,education.field_of_study ,business.industry ,Interstitial lung disease ,Retrospective cohort study ,Original Articles ,Pirfenidone ,Middle Aged ,respiratory system ,medicine.disease ,Fibrosis ,Combined pulmonary fibrosis and emphysema ,Idiopathic Pulmonary Fibrosis ,respiratory tract diseases ,3. Good health ,030228 respiratory system ,Disease Progression ,Female ,Lung Diseases, Interstitial ,business ,Progressive disease ,medicine.drug - Abstract
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, 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
- Published
- 2020
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