ArticleThe European respiratory journal2021
Progressive fibrosing interstitial lung disease: a clinical cohort (the PROGRESS study).
Article in The European respiratory journal, 2021. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 111 papers, 9 of them syntheses that pooled it.
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The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
111 citing papers in PubMed, 9 syntheses or guidelines pooled it, 215 citations in OpenAlex.
- Economic burden and cost drivers of interstitial lung disease: a systematic review.The European journal of health economics : HEPAC : health economics in prevention and care · 2026Pooled it
- Baseline total lung capacity and all-cause mortality in restrictive pulmonary disorders: a meta-analysis.BMC pulmonary medicine · 2025Pooled it
- Comorbidities in the idiopathic pulmonary fibrosis and progressive pulmonary fibrosis trial population: a systematic review and meta-analysis.European respiratory review : an official journal of the European Respiratory Society · 2025Pooled it
- Pooled it
- Mycophenolate and azathioprine efficacy in interstitial lung disease: a systematic review and meta-analysis.BMJ open respiratory research · 2024Pooled it
- The Role of Lung Microbiome in Fibrotic Interstitial Lung Disease-A Systematic Review.Biomolecules · 2024Pooled it
- Pooled it
- Guidelines of the Polish Respiratory Society on the Diagnosis and Treatment of Progressive Fibrosing Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis.Advances in respiratory medicine · 2022Guideline
- Meta-Analysis of Effect of Nintedanib on Reducing FVC Decline Across Interstitial Lung Diseases.Advances in therapy · 2022Pooled it
- Effect of nintedanib in patients with progressive pulmonary fibrosis associated with rheumatoid arthritis: data from the INBUILD trial.Clinical rheumatology · 2023Trial
- Weight loss and outcomes in subjects with progressive pulmonary fibrosis: data from the INBUILD trial.Respiratory research · 2023Trial
- Safety and tolerability of nintedanib in patients with progressive fibrosing interstitial lung diseases: data from the randomized controlled INBUILD trial.Respiratory research · 2022Trial
- Responsiveness and meaningful change thresholds of the Living with Pulmonary Fibrosis (L-PF) questionnaire Dyspnoea and Cough scores in patients with progressive fibrosing interstitial lung diseases.BMJ open respiratory research · 2022Trial
- Nintedanib in progressive interstitial lung diseases: data from the whole INBUILD trial.The European respiratory journal · 2022Trial
- Trajectories of progressive pulmonary fibrosis and outcomes in connective tissue disease associated interstitial lung disease (CTD-ILD).Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2026Article
- Cost-Effectiveness of Earlier Diagnosis of Fibrotic Interstitial Lung Diseases (Fibrotic ILDs) and Earlier Treatment of Its Progressive Form, Progressive Pulmonary Fibrosis (PPF).Advances in therapy · 2026Article
- Prevalence and Prognostic Impact of Progressive Pulmonary Fibrosis.Tuberculosis and respiratory diseases · 2026Review
- Progressive pulmonary fibrosis.European radiology · 2026Review
- A syndromic framework for progressive pulmonary fibrosis.Respiratory research · 2026Review
- Predictors of Progressive Fibrosing Interstitial Lung Diseases and Survival in Fibrosing Interstitial Lung Disease-Related Usual Interstitial Pneumonia.Medicina (Kaunas, Lithuania) · 2026Article
51 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
15 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
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<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.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.