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
. 2011 Feb 8:7:39-47.
doi: 10.2147/TCRM.S12209.

Pirfenidone treatment of idiopathic pulmonary fibrosis

Ye Gan  1 , Erica L Herzog, Richard H Gomer
Affiliations

Pirfenidone treatment of idiopathic pulmonary fibrosis

Ye Gan et al. Ther Clin Risk Manag. .

Abstract

Idiopathic pulmonary fibrosis (IPF) is a discrete clinicopathologic entity defined by the presence of usual interstitial pneumonia on high-resolution CT scan and/or open lung biopsy and the absence of an alternate diagnosis or exposure explaining these findings. There are currently no FDA-approved therapies available to treat this disease, and the 5-year mortality is ∼80%. The pyridone derivative pirfenidone has been studied extensively as a possible therapeutic agent for use in this deadly disease. This review will present the unique clinical features and management issues encountered by physicians caring for IPF patients, including the poor response to conventional therapy. The biochemistry and preclinical efficacy of pirfenidone will be discussed along with a comprehensive review of the clinical efficacy, safety, and side effects and patient-centered foci such as quality of life and tolerability. It is hoped that this information will lend insight into the complex issues surrounding the use of pirfenidone in IPF and lead to further investigation of this agent as a possible therapy in this devastating disease.

Keywords: clinical trials; fibrosis; pirfenidone.

PubMed Disclaimer

Figures

Figure 1
Figure 1
The structure of pirfenidone. After ingestion, the CH3 group at the bottom of the structure is rapidly metabolized to primarily COOH and to a lesser extent OH.
See this image and copyright information in PMC

References

    1. Wynn TA. Fibrotic disease and the T(H)1/T(H)2 paradigm. Nat Rev Immunol. 2004;4(8):583–594. - PMC - PubMed
    1. American Thoracic Society. European Respiratory Society American Thoracic Society/European Respiratory Society International Multidisciplinary Consensus Classification of the Idiopathic Interstitial Pneumonias. This joint statement of the American Thoracic Society (ATS), and the European Respiratory Society (ERS) was adopted by the ATS board of directors, 2001 Jun and by the ERS Executive Committee, 2001 Jun. Am J Respir Crit Care Med. 2002;165(2):277–304. - PubMed
    1. Raghu G, Weycker D, Edelsberg J, Bradford WZ, Oster G. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2006;174(7):810–816. - PubMed
    1. Yuksel M, Ozyurtkan MO, Bostanci K, Ahiskali R, Kodalli N. Acute exacerbation of interstitial fibrosis after pulmonary resection. Ann Thorac Surg. 2006;82(1):336–338. - PubMed
    1. Castelino FV, Goldberg H, Dellaripa PF. The impact of rheumatological evaluation in the management of patients with interstitial lung disease. Rheumatology (Oxford) 2010 Aug 4; Epub ahead of print. - PubMed