ArticleTherapeutic advances in respiratory disease
Pirfenidone in idiopathic pulmonary fibrosis: real-life experience in the referral centre of Siena.
Article in Therapeutic advances in respiratory disease. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers, 1 of them a synthesis that pooled it.
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Who cites it
18 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Real-world safety and effectiveness of pirfenidone and nintedanib in the treatment of idiopathic pulmonary fibrosis: a systematic review and meta-analysis.European journal of clinical pharmacology · 2024Pooled it
- Comparison of the Efficacy of Pirfenidone and Nintedanib in the Treatment of Patients with Idiopathic Pulmonary Fibrosis-A Single-Center Experience.Medicina (Kaunas, Lithuania) · 2026Article
- Pirfenidone in Idiopathic Pulmonary Fibrosis: Real-World Observation on Efficacy and Safety, Focus on Patients Undergoing Antithrombotic and Anticoagulant.Pharmaceuticals (Basel, Switzerland) · 2024Article
- Pirfenidone and Nintedanib in Pulmonary Fibrosis: Lights and Shadows.Pharmaceuticals (Basel, Switzerland) · 2024Review
- Disease progression in idiopathic pulmonary fibrosis under anti-fibrotic treatment.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2023Article
- Markers of Bronchiolitis Obliterans Syndrome after Lung Transplant: Between Old Knowledge and Future Perspective.Biomedicines · 2022Review
- Combined Sarcoidosis and Idiopathic Pulmonary Fibrosis (CSIPF): A New Phenotype or a Fortuitous Overlap? Scoping Review and Case Series.Journal of clinical medicine · 2022Article
- Article
- Mortality and survival in idiopathic pulmonary fibrosis: a systematic review and meta-analysis.ERJ open research · 2022Review
- Extracellular Vesicle Surface Signatures in IPF Patients: A Multiplex Bead-Based Flow Cytometry Approach.Cells · 2021Article
- Article
- Review
- Idiopathic pulmonary fibrosis patients with severe physiologic impairment: characteristics and outcomes.Respiratory research · 2021Article
- Assessing the Effectiveness of Pirfenidone in Idiopathic Pulmonary Fibrosis: Long-Term, Real-World Data from European IPF Registry (eurIPFreg).Journal of clinical medicine · 2020Article
- Metabolic Dysregulation in Idiopathic Pulmonary Fibrosis.International journal of molecular sciences · 2020Review
- A multicentre retrospective observational study on Polish experience of pirfenidone therapy in patients with idiopathic pulmonary fibrosis: the PolExPIR study.BMC pulmonary medicine · 2020Observational
- Long-Term Follow-Up of Patients With Idiopathic Pulmonary Fibrosis Treated With Pirfenidone or Nintedanib: A Real-Life Comparison Study.Frontiers in molecular biosciences · 2020Article
- Prognostic significance of forced vital capacity decline prior to and following antifibrotic therapy in idiopathic pulmonary fibrosis.Therapeutic advances in respiratory diseaseArticle
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Authors and funding
15 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundIdiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and has a median survival after diagnosis of 2-5 years. Pirfenidone is the first approved antifibrotic drug for the treatment of IPF. Here we report the functional progress, side effects and survival data of a population of patients with IPF, diagnosed at our centre and treated with pirfenidone.
methodsWe enrolled 91 patients with IPF (71 males) treated with pirfenidone. Clinical, survival and functional details were collected retrospectively at start of therapy and after 12, 24, 36 and 48 months of treatment. Lung function tests at least 12 months before starting therapy were available for 40 patients and were entered in the database, as well as side effects.
resultsDuring the observation period (922 ± 529 days), 27 patients died, 5 patients underwent lung transplant and 10 patients interrupted therapy due to adverse events or IPF progression. The median survival was 1606 days. There was a significant reduction in disease progression rate, as measured by trend of forced vital capacity, after 1 year of therapy with respect to before treatment (
conclusionsOur findings confirm the efficacy of pirfenidone in reducing functional progression of IPF and its excellent safety profile in a real-life setting. This study, designed on a long-term follow up, contributes to the growing evidence on safety, tolerability and efficacy of pirfenidone in IPF.
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