Cited by 454Interstitial Lung Diseases and Idiopathic Pulmonary FibrosisSystemic Sclerosis and Related DiseasesPulmonary Hypertension Research and Treatments

Antifibrotic activities of pirfenidone in animal models

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Schaefer, C.J., Ruhrmund, D.W., Pan, L., Seiwert, S.D., Kossen, K.·2011·European Respiratory Review

Abstract

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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Crossref · 10.1183/09059180.00001111

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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
Published
2011-06
Volume
20
Issue
120
Pages
85-97
ISSN
  • 0905-9180 (ISSN-L)
  • 1600-0617
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
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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