Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2022 Apr 14:8:e12.
doi: 10.15420/cfr.2021.30. eCollection 2022 Jan.

Pirfenidone for Idiopathic Pulmonary Fibrosis and Beyond

Alberto Aimo  1   2 , Giosafat Spitaleri  3 , Dario Nieri  4 , Laura Maria Tavanti  4 , Claudia Meschi  5 , Giorgia Panichella  1 , Josep Lupón  3   6   7 , Francesco Pistelli  4 , Laura Carrozzi  4   5 , Antoni Bayes-Genis  3   6 , Michele Emdin  1   2
Affiliations
Review

Pirfenidone for Idiopathic Pulmonary Fibrosis and Beyond

Alberto Aimo et al. Card Fail Rev. .

Abstract

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.

Keywords: Pirfenidone; animal models; clinical trials; heart; idiopathic pulmonary fibrosis; lung.

PubMed Disclaimer

Conflict of interest statement

Disclosure: The authors have no conflicts of interest to declare.

Figures

Figure 1:
Figure 1:. Cardiac Protective Effects of Pirfenidone
See this image and copyright information in PMC

Similar articles

See all similar articles

Cited by

See all "Cited by" articles

References

    1. Iyer SN, Wild JS, Schiedt MJ et al. Dietary intake of pirfenidone ameliorates bleomycin-induced lung fibrosis in hamsters. J Lab Clin Med. 1995;125:779–85. - PubMed
    1. Azuma A. Pirfenidone: antifibrotic agent for idiopathic pulmonary fibrosis. Expert Rev Respir Med. 2010;4:301–10. doi: 10.1586/ers.10.32. - DOI - PubMed
    1. Schaefer CJ, Ruhrmund DW, Pan L et al. Antifibrotic activities of pirfenidone in animal models. Eur Respir Rev. 2011;20:85–97. doi: 10.1183/09059180.00001111. - DOI - PMC - PubMed
    1. Raghu G, Rochwerg B, Zhang Y et al. An official ATS/ERS/JRS/ALAT clinical practice guideline: treatment of idiopathic pulmonary fibrosis. An update of the 2011 clinical practice guideline. Am J Respir Crit Care Med. 2015;192:e3–19. doi: 10.1164/rccm.201506-1063ST. - DOI - PubMed
    1. Travis WD, Costabel U, Hansell DM et al. An official American Thoracic Society/European Respiratory Society statement: update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188:733–48. doi: 10.1164/rccm.201308-1483ST. - DOI - PMC - PubMed

LinkOut - more resources