ArticleEBioMedicine2019
Endotype-phenotyping may predict a treatment response in progressive fibrosing interstitial lung disease.
Article in EBioMedicine, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 38 papers, 3 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
38 citing papers in PubMed, 3 syntheses or guidelines pooled it, 56 citations in OpenAlex.
- Impact of time to diagnosis in patients with progressive fibrotic ILD: a systematic review.BMJ open respiratory research · 2026Pooled it
- Pooled it
- Efficacy of Pirfenidone and Nintedanib in Interstitial Lung Diseases Other than Idiopathic Pulmonary Fibrosis: A Systematic Review.International journal of molecular sciences · 2023Pooled it
- Nintedanib in Patients With Autoimmune Disease-Related Progressive Fibrosing Interstitial Lung Diseases: Subgroup Analysis of the INBUILD Trial.Arthritis & rheumatology (Hoboken, N.J.) · 2022Trial
- Treatable traits approach for personalised management of sarcoidosis.BMJ open respiratory research · 2026Review
- Epidemiology and Prognosis of Progressive Pulmonary Fibrosis: A Literature Review.Pulmonary therapy · 2025Review
- Silencing IFIT3 suppresses the EGFR/VEGF pathway and modulates SOCS1 to attenuate skin fibrosis in systemic sclerosis.Apoptosis : an international journal on programmed cell death · 2025Article
- Korean Guidelines for the Diagnosis and Management of Interstitial Lung Diseases: Sarcoidosis.Tuberculosis and respiratory diseases · 2025Article
- Predictive Biomarkers and Novel Treatments for the Progressive Fibrosing Phenotype in Interstitial Lung Disease Associated with Connective Tissue Disease.Biomedicines · 2025Review
- Initiation of antifibrotic treatment in fibrosing interstitial lung disease: is the clock ticking till proven progression?European respiratory review : an official journal of the European Respiratory Society · 2025Review
- Article
- Rationale for phosphodiesterase-4 inhibition as a treatment strategy for interstitial lung diseases associated with rheumatic diseases.RMD open · 2024Review
- Compare three diagnostic criteria of progressive pulmonary fibrosis.Journal of thoracic disease · 2024Article
- Targeting Progression in Pulmonary Fibrosis: An Overview of Underlying Mechanisms, Molecular Biomarkers, and Therapeutic Intervention.Life (Basel, Switzerland) · 2024Review
- Walking the path of treatable traits in interstitial lung diseases.Respiratory research · 2023Article
- Sarcoidosis-associated pulmonary fibrosis: joining the dots.European respiratory review : an official journal of the European Respiratory Society · 2023Review
- Design of a phase III, double-blind, randomised, placebo-controlled trial of BI 1015550 in patients with progressive pulmonary fibrosis (FIBRONEER-ILD).BMJ open respiratory research · 2023Article
- "Management of myositis associated interstitial lung disease".Rheumatology international · 2023Review
- Progressive pulmonary fibrosis: an expert group consensus statement.The European respiratory journal · 2023Review
- The molecular mechanism underlying dermatomyositis related interstitial lung disease: evidence from bioinformatic analysis andFrontiers in immunology · 2023Article
Corrections and comments
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Authors and funding
10 authors at 2 institutions in 2 countries.
Funding
Abstract
backgroundSome interstitial lung disease (ILD) patients develop a progressive fibrosing-ILD phenotype (PF-ILD), with similar persistent lung function decline suggesting common molecular pathways involved. Nintedanib, a tyrosine kinase inhibitor targeting the PDGF, FGF, VEGF and M-CSF pathways, has shown comparable efficacy in idiopathic pulmonary fibrosis (IPF) and systemic sclerosis-associated ILD (SSc-ILD). We hypothesize that Nintedanib targeted molecular pathways will be augmented to a similar degree across PF-ILD regardless of aetiology.
methodsWe collected explanted lung tissue at the time of lung transplantation from 130 PF-ILD patients (99 (76%) IPF, 14 (11%) SSc-ILD, 17 (13%) other PF-ILD), and wedge biopsies from 200 donor lungs and measured PDGF, FGF, VEGF and M-CSF concentrations by Luminex.
findingsThe concentrations of PDGF-AA, PDGF-BB, FGF-2, VEGF and M-CSF were significantly increased in PF-ILD lungs compared to donor lungs (PDGF-AA 93·0 pg/ml [±97·2] vs. 37·5 pg/ml [±35·4], p < 0·001; PDGF-BB 102·5 pg/ml [±78·8] vs. 61·9 pg/ml [±47·0], p < 0·001; FGF-2 1442·4 pg/ml [±426·6] vs. 1201·7 pg/ml [±535·2], p = 0·009; VEGF 40·6 pg/ml [±20·1] vs. 24·9 pg/ml [±29·5], p < 0·001; and M-CSF 25526 pg/ml [±24,799] vs. 6120 pg/ml [±7245], p < 0·001). There were no significant differences in these growth factor/angiogenic molecules/cytokine concentrations when segregated by IPF, SSc-ILD and other PF-ILDs.
interpretationNintedanib specific targeted molecular pathways are augmented to a similar magnitude in all PF-ILD lung tissue as compared to controls, suggesting that Nintedanib treatment may be efficacious in PF-ILD regardless of aetiology. We speculate that clinical trials using Nintedanib for PF-ILD with or without IPF or SSc-ILD should show a similar relative reduction in FVC decline as seen in IPF and SSc-ILD (∼45-50%).
fundingHealth Grant P01-HL108793 (JAB), South-Eastern Norway Regional Health Authority Grant 2018072 (AMHV).
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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.