Antifibrotic activities of pirfenidone in animal models
Open accessAbstract
Pirfenidone is an orally active small molecule that has recently been evaluated in large clinical trials for the treatment of idiopathic pulmonary fibrosis, a fatal disease in which the uncontrolled deposition of extracellular matrix leads to progressive loss of lung function. This review describes the activity of pirfenidone in several well-characterised animal models of fibrosis in the lung, liver, heart and kidney. In these studies, treatment-related reductions in fibrosis are associated with modulation of cytokines and growth factors, with the most commonly reported effect being reduction of transforming growth factor-β. The consistent antifibrotic activity of pirfenidone in a broad array of animal models provides a strong preclinical rationale for the clinical characterisation of pirfenidone in pulmonary fibrosis and, potentially, other conditions with a significant fibrotic component.
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Pirfenidone is a small molecule that modulates fibrotic and inflammatory pathways, including TGF-beta and TNF-alpha.
“Pirfenidone is a small molecule that modulates fibrotic and inflammatory pathways, including TGF-β and TNF-α.9,10”
From Deupirfenidone Compared with Pirfenidone and Placebo in Idiopathic Pulmonary Fibrosis (ELEVATE-IPF): a Phase 2b Randomized Placebo-Controlled Trial (2026) · cites this paper as [10]
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- Authors
- Schaefer, C. J., Ruhrmund, D. W., Pan, L., Seiwert, S. D., & Kossen, K.
- ORCID
- Pan, L.
- Venue
- European Respiratory Review
- Publisher
- European Respiratory Society (ERS)
- Published
- 2011-06
- Volume
- 20
- Issue
- 120
- Pages
- 85-97
- ISSN
- 0905-9180 (ISSN-L)
- 1600-0617
- PMC
- PMC9487788
- OpenAlex
- W2135863640
- Cited by
- 454
- References
- 94
- Citations by year
- 2026: 20 · 2025: 27 · 2024: 32 · 2023: 30 · 2022: 49 · 2021: 26 · 2020: 37 · 2019: 32 · 2018: 37 · 2017: 33 · 2016: 32 · 2015: 35 · 2014: 36 · 2013: 16 · 2012: 8
- FWCI
- 3.29
- Type
- journal article
- Language
- en
- Open access
- diamond
- doi.org
- Locations
- European Respiratory Review (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Health Sciences › Medicine › Pathology and Forensic Medicine › Systemic Sclerosis and Related Diseases
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Keywords
- pirfenidone, antifibrotic activity, idiopathic pulmonary fibrosis, liver fibrosis, myocardial fibrosis, renal fibrosis, extracellular matrix deposition, TGF-beta, cytokines, growth factors
- MeSH terms
- Administration, Oral, Animals, Disease Models, Animal, Humans, Kidney Diseases, Liver Cirrhosis, Cardiomyopathies, Pulmonary Fibrosis, Pyridones, Signal Transduction, Transforming Growth Factor beta, Extracellular Matrix Proteins
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