ArticleJournal of clinical medicine2020
Assessing the Effectiveness of Pirfenidone in Idiopathic Pulmonary Fibrosis: Long-Term, Real-World Data from European IPF Registry (eurIPFreg).
Article in Journal of clinical medicine, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 19 citations in OpenAlex.
- Tidal Volume in Idiopathic Pulmonary Fibrosis: Restriction Severity and Prognostic Information beyond Forced Vital Capacity.Respiration; international review of thoracic diseases · 2026Article
- Peri-transplant frailty changes impact survival in interstitial lung diseases.ERJ open research · 2026Article
- Pexidartinib and Nintedanib Combination Therapy Targets Macrophage Polarization to Reverse Pulmonary Fibrosis: A Preclinical Study.International journal of molecular sciences · 2025Article
- Lung Function Course of Patients With Pulmonary Fibrosis After Initiation of Anti-Fibrotic Treatment: Real-World Data From the Dutch National Registry.Respirology (Carlton, Vic.) · 2025Article
- Protocol and research program of the European registry and biobank for interstitial lung diseases (eurILDreg).BMC pulmonary medicine · 2024Article
- Compare three diagnostic criteria of progressive pulmonary fibrosis.Journal of thoracic disease · 2024Article
- Improved Survival of IPF patients Treated With Antifibrotic Drugs Compared With Untreated Patients.Lung · 2023Observational
- A randomized controlled phase III trial protocol: perioperative pirfenidone therapy in patients with non-small cell lung cancer combined with idiopathic pulmonary fibrosis to confirm the preventative effect against postoperative acute exacerbation: the PIII-PEOPLE study (NEJ034).Journal of thoracic disease · 2023Article
- Telomere length associates with chronological age and mortality across racially diverse pulmonary fibrosis cohorts.Nature communications · 2023Article
- Article
- Soluble ST2 in serum predicts the prognosis of idiopathic pulmonary fibrosis: a retrospective study.Annals of translational medicine · 2022Article
- Targeting Histone Deacetylases in Idiopathic Pulmonary Fibrosis: A Future Therapeutic Option.Cells · 2022Review
- Analysis of the safety and efficacy of different plasma concentrations of pirfenidone in patients with idiopathic pulmonary fibrosis.Frontiers in pharmacology · 2022Article
- A scalable 3D tissue culture pipeline to enable functional therapeutic screening for pulmonary fibrosis.APL bioengineering · 2021Article
Corrections and comments
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Authors and funding
9 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundIdiopathic pulmonary fibrosis (IPF) is a chronic progressive fibrotic pulmonary disease with rising incidence. In this study the effectiveness of pirfenidone, as measured by longitudinal change in individual slope of forced vital capacity (FVC) prior to and after initiating pirfenidone treatment, was evaluated in IPF patients recruited into the European registry for idiopathic pulmonary fibrosis (eurIPFreg). Secondary variables were the evaluation of the change in individual slope of diffusion capacity of the lungs for carbon monoxide (DLco), the Borg dyspnea scale, and six-minute walking distance (6MWD), as well as survival analyses.
resultsData of 122 eurIPFreg patients, who had at least two pulmonary function tests (PFTs) prior to or under treatment with pirfenidone, were analyzed by calculating slope-changes. The global analysis revealed an average slope change of +1.48 ± 0.28 (% per annum (p.a)) after start of treatment (
conclusionsWe report the effectiveness of pirfenidone in a European "real world" IPF cohort with outcome data extending up to 9 years. Global analyses demonstrated a positive effect of pirfenidone on the decline of the lung function over time. Survival was dependent on Gender-Age-Physiology (GAP) score and age prior to therapy.
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Registered trials
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