Pirfenidone for Idiopathic Pulmonary Fibrosis and Beyond
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...
| Main Authors: | , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Radcliffe Medical Media
2022-04-01
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| Series: | Cardiac Failure Review |
| Online Access: | https://www.cfrjournal.com/articleindex/cfr.2021.30 |
| _version_ | 1797199975854112768 |
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| author | Alberto Aimo Giosafat Spitaleri Dari Nieri Laura Maria Tavanti Claudia Meschi Giorgia Panichella Josep Lupón Francesco Pistelli Laura Carrozzi Antoni Bayes-Genis Michele Emdin |
| author_facet | Alberto Aimo Giosafat Spitaleri Dari Nieri Laura Maria Tavanti Claudia Meschi Giorgia Panichella Josep Lupón Francesco Pistelli Laura Carrozzi Antoni Bayes-Genis Michele Emdin |
| author_sort | Alberto Aimo |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-07T17:39:50Z |
| format | Article |
| id | doaj.art-41f6037c08aa46eeb1f7ff900fcbd967 |
| institution | Directory Open Access Journal |
| issn | 2057-7540 2057-7559 |
| language | English |
| last_indexed | 2024-04-24T07:24:18Z |
| publishDate | 2022-04-01 |
| publisher | Radcliffe Medical Media |
| record_format | Article |
| series | Cardiac Failure Review |
| spelling | doaj.art-41f6037c08aa46eeb1f7ff900fcbd9672024-04-20T16:02:49ZengRadcliffe Medical MediaCardiac Failure Review2057-75402057-75592022-04-01810.15420/cfr.2021.30Pirfenidone for Idiopathic Pulmonary Fibrosis and BeyondAlberto Aimo0Giosafat Spitaleri1Dari Nieri2Laura Maria Tavanti3Claudia Meschi4Giorgia Panichella5Josep Lupón6Francesco Pistelli7Laura Carrozzi8Antoni Bayes-Genis9Michele Emdin10Institute of Life Sciences, Scuola Superiore Sant’Anna, Pisa, Italy; Fondazione Toscana Gabriele Monasterio, Pisa, ItalyHeart Failure Clinic and Cardiology Service, University Hospital Germans Trias i Pujol, Badalona, SpainPulmonary Unit, Cardiothoracic and Vascular Department, Pisa University Hospital, Pisa, ItalyPulmonary Unit, Cardiothoracic and Vascular Department, Pisa University Hospital, Pisa, ItalyDepartment of Surgical, Medical and Molecular Pathology and Critical Care Medicine, University of Pisa, Pisa, ItalyInstitute of Life Sciences, Scuola Superiore Sant’Anna, Pisa, ItalyHeart Failure Clinic and Cardiology Service, University Hospital Germans Trias i Pujol, Badalona, Spain; Department of Medicine, Universitat Autònoma de Barcelona, Barcelona, Spain; Centro de Investigación Biomédica en Red de Enfermedades Cardiovasculares (CIBERCV), Instituto de Salud Carlos III, Madrid, SpainPulmonary Unit, Cardiothoracic and Vascular Department, Pisa University Hospital, Pisa, ItalyPulmonary Unit, Cardiothoracic and Vascular Department, Pisa University Hospital, Pisa, Italy; Department of Surgical, Medical and Molecular Pathology and Critical Care Medicine, University of Pisa, Pisa, ItalyHeart Failure Clinic and Cardiology Service, University Hospital Germans Trias i Pujol, Badalona, Spain; Department of Medicine, Universitat Autònoma de Barcelona, Barcelona, SpainInstitute of Life Sciences, Scuola Superiore Sant’Anna, Pisa, Italy; Fondazione Toscana Gabriele Monasterio, Pisa, ItalyPirfenidone (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.https://www.cfrjournal.com/articleindex/cfr.2021.30 |
| spellingShingle | Alberto Aimo Giosafat Spitaleri Dari Nieri Laura Maria Tavanti Claudia Meschi Giorgia Panichella Josep Lupón Francesco Pistelli Laura Carrozzi Antoni Bayes-Genis Michele Emdin Pirfenidone for Idiopathic Pulmonary Fibrosis and Beyond Cardiac Failure Review |
| title | Pirfenidone for Idiopathic Pulmonary Fibrosis and Beyond |
| title_full | Pirfenidone for Idiopathic Pulmonary Fibrosis and Beyond |
| title_fullStr | Pirfenidone for Idiopathic Pulmonary Fibrosis and Beyond |
| title_full_unstemmed | Pirfenidone for Idiopathic Pulmonary Fibrosis and Beyond |
| title_short | Pirfenidone for Idiopathic Pulmonary Fibrosis and Beyond |
| title_sort | pirfenidone for idiopathic pulmonary fibrosis and beyond |
| url | https://www.cfrjournal.com/articleindex/cfr.2021.30 |
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