ArticleThe European respiratory journal2022
Lung function trajectory in progressive fibrosing interstitial lung disease.
Article in The European respiratory journal, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 78 papers, 2 of them syntheses that pooled it.
What it found
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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
78 citing papers in PubMed, 2 syntheses or guidelines pooled it, 120 citations in OpenAlex.
- Effect of breathing exercises on patients with interstitial lung disease: A systematic review and meta-analysis.Quality of life research : an international journal of quality of life aspects of treatment, care and rehabilitation · 2024Pooled 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
- Zinpentraxin Alfa for Idiopathic Pulmonary Fibrosis: The Randomized Phase III STARSCAPE Trial.American journal of respiratory and critical care medicine · 2024Trial
- Cough in Interstitial Lung Disease: Current Knowledge and Research Gaps.Pulmonary therapy · 2026Review
- Progressive pulmonary fibrosis criteria predict mortality in treated idiopathic pulmonary fibrosis: a prospective cohort study.BMC pulmonary medicine · 2026Article
- Prevalence and Prognostic Impact of Progressive Pulmonary Fibrosis.Tuberculosis and respiratory diseases · 2026Review
- Prognostic Value of Bronchoalveolar Lavage in Systemic Autoimmune Rheumatic Diseases-Associated Interstitial Lung Disease.Journal of clinical medicine · 2026Article
- Real-World Experience with Nintedanib in a Progressive Pulmonary Fibrosis Cohort.Medicina (Kaunas, Lithuania) · 2026Observational
- Progressive pulmonary fibrosis.European radiology · 2026Review
- ILDMDB: a manually curated database of metabolite-disease associations in interstitial lung diseases.Metabolomics : Official journal of the Metabolomic Society · 2026Article
- A syndromic framework for progressive pulmonary fibrosis.Respiratory research · 2026Review
- Molecular endotypes predict differential response to immunosuppressant therapy in non-idiopathic pulmonary fibrosis interstitial lung disease.American journal of respiratory and critical care medicine · 2026Observational
- Prioritizing Therapeutic Targets for Interstitial Lung Disease: A Causal Mediation Analysis.Research square · 2026Article
- Nintedanib for progressive pulmonary fibrosis in real-world setting: an observational study comparing outcomes with an IPF cohort.BMC pulmonary medicine · 2026Observational
- Serum metabolic characteristics of interstitial lung disease: a potential link between endocrine aging and gut-derived metabolites.Frontiers in medicine · 2026Article
- Circulating biomarkers in subjects with progressive pulmonary fibrosis: data from the INBUILD trial.ERJ open research · 2026Article
- The dual promise of extracellular vesicles in lung diseases: towards reliable biomarkers and drug delivery vectors.European respiratory review : an official journal of the European Respiratory Society · 2026Review
- Progressive pulmonary fibrosis: the importance of identification and intervention.European respiratory review : an official journal of the European Respiratory Society · 2026Review
- A multidimensional index with SpORespiratory research & clinical practice · 2026Article
- Associations of interstitial lung disease subtype and CT pattern with lung function and survival.Thorax · 2025Article
18 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
16 authors at 7 institutions in 2 countries.
Funding
Abstract
backgroundProposed criteria for progressive fibrosing interstitial lung disease (PF-ILD) have been linked to increased mortality risk, but lung function trajectory after satisfying individual criteria remains unknown. Because survival is rarely employed as the primary end-point in therapeutic trials, identifying PF-ILD criteria that best predict subsequent change in forced vital capacity (FVC) could improve clinical trial design.
methodsA retrospective, multicentre longitudinal cohort analysis was performed in consecutive patients with fibrotic connective tissue disease-associated ILD (CTD-ILD), chronic hypersensitivity pneumonitis and idiopathic interstitial pneumonia at three US centres (test cohort) and one UK centre (validation cohort). 1-year change in FVC after satisfying proposed PF-ILD criteria was estimated using joint modelling. Subgroup analyses were performed to determine whether results varied across key subgroups.
results1227 patients were included, with CTD-ILD predominating. Six out of nine PF-ILD criteria were associated with differential 1-year change in FVC, with radiological progression of fibrosis, alone and in combination with other features, associated with the largest subsequent decline in FVC. Findings varied significantly by ILD subtype, with CTD-ILD demonstrating little change in FVC after satisfying most PF-ILD criteria, while other ILDs showed significantly larger changes. Findings did not vary after stratification by radiological pattern or exposure to immunosuppressant therapy. Near-term change in FVC after satisfying proposed PF-ILD criteria was heterogeneous depending on the criterion assessed and was strongly influenced by ILD subtype.
conclusionsThese findings may inform future clinical trial design and suggest ILD subtype should be taken into consideration when applying PF-ILD criteria.
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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.