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

Swati Gulati,1 Tracy R Luckhardt1,2 1Division of Pulmonary, Allergy, and Critical Care Medicine, University of Alabama at Birmingham, Birmingham, AL, USA; 2Department of Medicine, University of Alabama at Birmingham, Birmingham, AL, USACorrespondence: Swati GulatiDivision of Pulmonary, Allergy, and Critical Care Medicine, University of Alabama at Birmingham, 1900 University Blvd THT 541E, Birmingham, AL 35294-2180, USATel +1 205 975 6376Fax +1 205 934 1721Email sgulati@uabmc.eduAbstract: 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, idiopathic pulmonary fibrosis, efficacy, safety, tolerability