Observational studyTherapeutic advances in respiratory disease
Real-life experiences in a single center: efficacy of pirfenidone in idiopathic pulmonary fibrosis and fibrotic idiopathic non-specific interstitial pneumonia patients.
Observational study 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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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
18 citing papers in PubMed, 1 synthesis or guideline pooled it, 20 citations in OpenAlex.
- 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
- Acute exacerbation in fibrotic interstitial lung disease: an international working group report.American journal of respiratory and critical care medicine · 2026Review
- Antifibrotic therapy for Lower Urinary Tract Symptoms secondary to Benign Prostatic Hyperplasia.Prostate cancer and prostatic diseases · 2026Review
- Review
- The Fibrotic-Cancer Continuum in IPF: Shared Mechanisms, Clinical Implications and Therapeutic Challenges.Life (Basel, Switzerland) · 2026Review
- Antifibrotic Treatment in Idiopathic Pulmonary Fibrosis (IPF) and Progressive Pulmonary Fibrosis (PPF): Real-World outcomes from a Single-Center study.Lung India : official organ of Indian Chest Society · 2026Article
- Progress in the Application of Pirfenidone in Post-COVID-19 Pulmonary Fibrosis: A Review.Cureus · 2025Review
- Korean Guidelines for Diagnosis and Management of Idiopathic Nonspecific Interstitial Pneumonia.Tuberculosis and respiratory diseases · 2025Article
- Prolonged corticosteroid therapy and lung abnormalities in patients after severe COVID-19 pneumonia.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2024Article
- Real-World Experience in the Clinical Use of Pirfenidone in Patients with Idiopathic Pulmonary Fibrosis in Taiwan: A Post-Marketing Surveillance Study.Biomedicines · 2024Article
- Article
- Disease progression in idiopathic pulmonary fibrosis under anti-fibrotic treatment.Sarcoidosis, vasculitis, and diffuse lung diseases : official journal of WASOG · 2023Article
- The influence of immortal time bias in observational studies examining associations of antifibrotic therapy with survival in idiopathic pulmonary fibrosis: A simulation study.Frontiers in medicine · 2023Article
- Pirfenidone inhibits cell fibrosis in connective tissue disease-associated interstitial lung disease by targeting the TNF-α/STAT3/KL6 pathway.Journal of thoracic disease · 2022Article
- Idiopathic pulmonary fibrosis: Current and future treatment.The clinical respiratory journal · 2022Review
- Analysis of the safety and efficacy of different plasma concentrations of pirfenidone in patients with idiopathic pulmonary fibrosis.Frontiers in pharmacology · 2022Article
- Molecular Mechanisms and Cellular Contribution from Lung Fibrosis to Lung Cancer Development.International journal of molecular sciences · 2021Review
- Myofibroblasts: Function, Formation, and Scope of Molecular Therapies for Skin Fibrosis.Biomolecules · 2021Review
Corrections and comments
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundPirfenidone is the first antifibrotic drug approved for the treatment of idiopathic pulmonary fibrosis (IPF) and it is used in the treatment of other interstitial pneumonias, such as unclassifiable interstitial lung disease (ILD) and connective tissue-related ILD. This study examined the efficacy of pirfenidone in patients with IPF and fibrotic idiopathic non-specific interstitial pneumonia (f-iNSIP).
methodsIn a retrospective real-life study, 67 IPF and 24 f-iNSIP patients were enrolled and classified into a pirfenidone group and a non-antifibrotic group. The level of forced vital capacity (FVC) and diffusing capacity of lung for carbon monoxide (DLco) at baseline, 6, 12 and 24 months were recorded. The level of KL-6 in serum was detected by chemiluminescence enzyme immunoassay (CLEIA). The prognosis and safety outcomes were collected from patients.
resultsIn IPF patients, pirfenidone decreased the mean change of FVC and DLco, and decreased the proportion of patients with a ⩾10% decline in FVC or a ⩾15% decline in DLco compared with the non-antifibrotic group. There was no difference in the mean change of FVC and DLco between smokers and non-smokers who received pirfenidone treatment. There was an improvement in progression-free survival, defined as the time to the first occurrence of acute exacerbation or death related to pulmonary fibrosis. Moreover, the ratio of patients who experienced acute exacerbation and death related to pulmonary fibrosis was lower in the pirfenidone group. There was no change in lung function and prognosis between the pirfenidone and non-antifibrotic groups in f-iNSIP patients. The KL-6 level slightly decreased after 1 year of pirfenidone treatment but not significantly. Gastrointestinal and skin-related adverse events were most common, and four patients ceased treatment due to the side effects.
conclusionsPirfenidone safely reduced disease progression by improving the lung function and progression-free survival in IPF patients, with acceptable side effects. The efficacy of pirfenidone on f-iNSIP was not significant, suggesting the need for further studies.
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