Pirfenidone for idiopathic pulmonary fibrosis and beyond
Open accessAbstract
Pirfenidone (PFD) slows the progression of idiopathic pulmonary fibrosis (IPF) by inhibiting the exaggerated fibrotic response and possibly through additional mechanisms, such as anti-inflammatory effects. PFD has also been evaluated in other fibrosing lung diseases. Myocardial fibrosis is a common feature of several heart diseases and the progressive deposition of extracellular matrix due to a persistent injury to cardiomyocytes may trigger a vicious cycle that leads to persistent structural and functional alterations of the myocardium. No primarily antifibrotic medications are used to treat patients with heart failure. There is some evidence that PFD has antifibrotic actions in various animal models of cardiac disease and a phase II trial on patients with heart failure and preserved ejection fraction has yielded positive results. This review summarises the evidence about the possible mechanisms of IPF and modulation by PFD, the main results about IPF or non-IPF interstitial pneumonias and also data about PFD as a potential protective cardiac drug.
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The current standard-of-care therapy regimen for IPF includes either nintedanib or pirfenidone.
“The current standard-of-care (SOC) therapy regimen to treat IPF includes either nintedanib, a broadly acting inhibitor of receptor tyrosine kinases31, or pirfenidone, an inhibitor of TGFβ-mediated fibroblast-to-myofibroblast transition32.”
From A generative AI-discovered TNIK inhibitor for idiopathic pulmonary fibrosis: a randomized phase 2a trial (2025) · cites this paper as [32]
Pre-clinical studies demonstrated that pirfenidone exerts inhibitory effects on inflammatory, antioxidant and profibrotic pathways, such as fibroblast proliferation, TGFβ1 driven myofibroblast transformation and pro-inflammatory cytokine release.
“pre-clinical studies demonstrated that it exerts inhibitory effects on inflammatory, antioxidant and profibrotic pathways, such as fibroblast proliferation, TGFβ1 driven myofibroblast transformation and pro-inflammatory cytokine release (Aimo et al., 2022).”
From Pulmonary fibrosis: Emerging diagnostic and therapeutic strategies (2023) · cites this paper as Aimo et al., 2022
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References 90 · Cited by 88
- Authors
- Aimo, A., Spitaleri, G., Nieri, D., Tavanti, L. M., Meschi, C., Panichella, G., Lupón, J., Pistelli, F., Carrozzi, L., Bayes-Genis, A., & Emdin, M.
- Affiliations
- Scuola Superiore Sant'Anna (IT)
- Hospital Universitari Germans Trias i Pujol (ES)
- Pulmonary Unit, Cardiothoracic and Vascular Department, Pisa University Hospital, Pisa, Italy
- Azienda Ospedaliera Universitaria Pisana (IT)
- University of Pisa (IT)
- Universitat Autònoma de Barcelona (ES)
- Heart Failure Clinic and Cardiology Service, University Hospital Germans Trias i Pujol, Badalona, Spain; Department of Medicine, Universitat Autònoma de Barcelona, Barcelona, Spain
- Corresponding author
- Aimo, Alberto
- ORCID
- Venue
- Cardiac Failure Review
- Publisher
- Radcliffe Medical Media Ltd
- Published
- 2022-04-14
- Volume
- 8
- Pages
- e12-e12
- ISSN
- 2057-7540 (ISSN-L)
- 2057-7559
- PMC
- PMC9062707
- OpenAlex
- W4223999663
- Cited by
- 88
- References
- 90
- Citations by year
- 2026: 16 · 2025: 33 · 2024: 19 · 2023: 14 · 2022: 5
- FWCI
- 3.07
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Licence
- Locations
- Cardiac failure review (publishedVersion, cc-by-nc)
- PubMed Central (publishedVersion, cc-by-nc)
- PubMed (publishedVersion)
- CINECA IRIS Institutial research information system (University of Pisa) (submittedVersion)
- Dipòsit Digital de Documents de la UAB (Universitat Autònoma de Barcelona) (submittedVersion, cc-by-nc)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion)
- CINECA IRIS Institutional Research Information System (Sant'Anna School of Advanced Studies) (submittedVersion, public-domain)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Pulmonary Hypertension Research and Treatments
- Health Sciences › Medicine › Cardiology and Cardiovascular Medicine › Cardiac Fibrosis and Remodeling
- Keywords
- pirfenidone, idiopathic pulmonary fibrosis, antifibrotic therapy, myocardial fibrosis, heart failure with preserved ejection fraction, interstitial lung disease, extracellular matrix deposition, cardiomyocyte injury, anti-inflammatory activity, fibrotic response
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