A Review of Pirfenidone as an Anti-Fibrotic in Idiopathic Pulmonary Fibrosis and Its Probable Role in Other Diseases

Our plain-English summary of A Review of Pirfenidone as an Anti-Fibrotic in Idiopathic Pulmonary Fibrosis and Its Probable Role in Other Diseases, by P.V. Shah, P. Balani, A.R. Lopez, et al (Curēus, 2021).

The study

• Authors: P.V. Shah, P. Balani, A.R. Lopez, C.M.N. Nobleza, M. Siddiqui, S. Khan
• Journal: Curēus
• Published: 2021;13(1):e12482
• Type of article: Review
• Cited by: 40 articles, as counted by NIH iCite
• Read it: PubMed · Publisher · Free full text

Abstract

Fibrosis is the result of chronic inflammation and is known to pathologically occur in many organs and systems. Pirfenidone (PFD) is an anti-fibrotic known for its use in idiopathic pulmonary fibrosis (IPF). In addition to being an anti-fibrotic, it acts as an anti-inflammatory and antioxidant as well. There have been studies on PFD in other diseases, some clinical and others preclinical. We have compiled and reviewed them to highlight just how widespread PFD use could be. Among many benefits of PFD in IPF, PFD has effectively improved patients' survival in those who had an acute exacerbation of IPF and has reduced respiratory-related hospitalization, among few others. PFD also has shown an improvement in vital capacity in patients with chronic hypersensitive pneumonitis. Also, it has demonstrated anti-fibrotic effects in systemic sclerosis-associated interstitial lung disease. In other diseases outside the lungs, PFD has reversed insulin resistance and proven to be effective in non-alcoholic steatohepatitis (NASH). It has prevented blindness post-alkali injury to the eye and has proven to decrease the proliferation of mesothelioma cells, just to name a few. This review encourages further research in connection with PFD and its use in other diseases and PFD pros in IPF.

Copyright © 2021, Shah et al.

In plain English

This article was a review, not a new study. The authors compiled published research on pirfenidone, an anti-fibrotic drug used for idiopathic pulmonary fibrosis (IPF), and on its testing in other diseases. Many of those studies were animal or laboratory studies. For IPF, one reviewed post hoc analysis reported significantly lower respiratory-related hospitalization with pirfenidone than with placebo. One study of IPF patients hospitalized for an acute flare-up compared 11 patients on pirfenidone with nine controls and reported improved survival. One retrospective cohort reported a 30% survival benefit at three years. Among other diseases, the review described a study in malignant mesothelioma. In that study, pirfenidone decreased the proliferation and migration of mesothelioma cells and altered the mesothelioma tumor microenvironment. The article did not discuss asbestos and did not say whether the mesothelioma studied was pleural or peritoneal. The authors noted that many IPF studies were post hoc analyses with small samples, drawn mostly from men and white patients in the ASCEND and CAPACITY trials. The authors concluded that pirfenidone has a vast scope and called for further studies.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Narrative review of published clinical, animal and in vitro studies; no search method is described
• Who was studied: No patients of its own; the review covered published studies of pirfenidone in IPF and in other diseases, including animal and in vitro studies
• Exposure: The drug pirfenidone (PFD), an anti-fibrotic; no asbestos or fiber exposure was studied
• Main results:
  • In a reviewed post hoc analysis (Ley et al.) of IPF patients, the pirfenidone group had significantly lower respiratory-related hospitalization than the placebo group
  • In a reviewed study (Vianello et al.) of IPF patients hospitalized for acute exacerbation, 11 patients on pirfenidone were compared with nine controls, and improved survival was reported; the sample size was not large
  • In a reviewed retrospective cohort (Margaritopoulos et al.) without strict selection criteria, the three-year survival rate increased in the pirfenidone group, with a 30% survival benefit
  • In a reviewed post hoc study (Nathan et al.) of 170 patients from the ASCEND and CAPACITY trials with more advanced lung function impairment, those receiving pirfenidone had a decreased risk of all-cause mortality and less lung function decline than those on placebo
  • A reviewed study (Li et al.) was done in malignant mesothelioma; the site (pleural or peritoneal) is not stated
  • In that study, pirfenidone decreased the migration and proliferation of mesothelioma cells and altered the mesothelioma tumor microenvironment, through reduced ERK and AKT pathways
  • In a reviewed study of 63 patients with systemic sclerosis-interstitial lung disease, adverse events were more common with a two-week titration period than with a four-week titration period
  • In a reviewed study of chronic hypersensitive pneumonitis, vital capacity improved significantly in the six months after starting pirfenidone compared with the six months before
• The authors' conclusion: The authors concluded that, despite the limitations, the reviewed studies indicate pirfenidone has a vast scope, and that future studies could change outcomes in many diseases
• Limitations the authors state:
  • Animal and in vitro studies were included in the review
  • A few of the reviewed studies were more than five years old
  • Several reviewed IPF studies had mainly men and white participants and small samples, which limits generalizability
  • Patients in the ASCEND and CAPACITY trial-based studies were chosen under strict inclusion and exclusion criteria

Topics

Medical terms in this study, explained in our Lexicon: Idiopathic, Pulmonary Fibrosis, Fibrosis, Chronic Inflammation, Pneumonitis, Interstitial Lung Disease, Mesothelioma.

Related studies

• The Its Not JUST Idiopathic pulmonary fibrosis Study (INJUSTIS): description of the protocol for a multicentre prospective observational cohort study identifying biomarkers of progressive fibrotic lung disease — F. Khan, I. Stewart, et al. (2019)
• Molecular Systems Architecture of Fibrotic Lung Microenvironment in Idiopathic Pulmonary Fibrosis — V.A.S. Ayyadurai, Y. Manoharan, et al. (2026)
• Relative release of interleukin-1 beta and interleukin-1 receptor antagonist by alveolar macrophages. A study in asbestos-induced lung disease, sarcoidosis, and idiopathic pulmonary fibrosis — J.N. Kline, D.A. Schwartz, et al. (1993)
• The role of inflammation in the pathogenesis of idiopathic pulmonary fibrosis — B.D. Bringardner, C.P. Baran, et al. (2008)
• The epithelium in idiopathic pulmonary fibrosis: breaking the barrier — A. Camelo, R. Dunmore, et al. (2014)
• A role for the receptor for advanced glycation end products in idiopathic pulmonary fibrosis — J.M. Englert, L.E. Hanford, et al. (2008)

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