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
. 2020 May 7:12:85-94.
doi: 10.2147/DHPS.S224007. eCollection 2020.

Updated Evaluation of the Safety, Efficacy and Tolerability of Pirfenidone in the Treatment of Idiopathic Pulmonary Fibrosis

Affiliations
Review

Updated Evaluation of the Safety, Efficacy and Tolerability of Pirfenidone in the Treatment of Idiopathic Pulmonary Fibrosis

Swati Gulati et al. Drug Healthc Patient Saf. .

Abstract

Idiopathic Pulmonary Fibrosis (IPF) is a chronic fibrotic disease characterized by a progressive decline in lung function with a median survival of 3-5 years after diagnosis. The course of disease is highly variable and unpredictable, often punctuated by episodes of acute respiratory failure, known as acute exacerbations. The incidence of IPF is on the rise due to the aging population, as age is the most important risk factor for this disease. Pirfenidone and nintedanib are the two anti-fibrotic drugs approved for IPF which have shown reduction in lung function decline. This review will discuss the efficacy, safety and tolerability profile of pirfenidone from clinical trials and the real-world clinical experience. Pirfenidone reduces the decline in lung function and improves progression-free survival in patients with IPF. It is generally well tolerated with the most common side effects being gastrointestinal and phototoxicity.

Keywords: Pirfenidone; efficacy; idiopathic pulmonary fibrosis; safety; tolerability.

PubMed Disclaimer

Conflict of interest statement

Tracy R Luckhardt reports personal fees from Boehringer Ingelheim and France Foundation, outside the submitted work. The authors report no other conflicts of interest in this work.

References

    1. Raghu G, Remy-Jardin M, Myers JL, et al. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018;198(5):e44–e68. doi:10.1164/rccm.201807-1255ST - DOI - PubMed
    1. Raghu G, Chen S-Y, Yeh W-S, et al. Idiopathic pulmonary fibrosis in US medicare beneficiaries aged 65 years and older: incidence, prevalence, and survival, 2001-11. Lancet Respir Med. 2014;2(7):566–572. doi:10.1016/S2213-2600(14)70101-8 - DOI - PubMed
    1. Ley B, Collard HR, King TE. Clinical course and prediction of survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2011;183(4):431–440. doi:10.1164/rccm.201006-0894CI - DOI - PubMed
    1. Collard HR, Ryerson CJ, Corte TJ, et al. Acute exacerbation of idiopathic pulmonary fibrosis. An international working group report. Am J Respir Crit Care Med. 2016;194(3):265–275. doi:10.1164/rccm.201604-0801CI - DOI - PubMed
    1. Noble PW, Albera C, Bradford WZ, et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials. Lancet Lond Engl. 2011;377(9779):1760–1769. doi:10.1016/S0140-6736(11)60405-4 - DOI - PubMed