Pirfenidone Experience in Idiopathic Pulmonary Fibrosis at a Tertiary Hospital in Oman

Nasser Busaidi

doi:10.31579/2693-2156/001

Abstract

Objective: The aim of this study is to evaluate the clinical and safety profile of Pirfenidone treatment in IPF. Methodology: This is a retrospective analysis of prospectively collected data via electronic system called “Al Shifa”. Data of all patients, who received Pirfenidone at the Royal Hospital in the period between April 2012 to April 2017, were retrieved into an excel sheet. The data retrieved at two time points: before starting the drug and last readings at time of the study. Results: There were 90 patients with IPF that were treated with Pirfenidone, The age ranges between 35 to 91 years old. All patient assessed by Forced Vital Capacity (FVC) by spirometry, and 6-minutes’ walk test (6MWT). The mean percentage of pre- FVC (before starting the drug) was 59.3%, while follow-up FVC (the last reading at the time of entering the data) was 60.3%. The Pre-6MWT test; the mean of distance they manage to walk in 6 mints, was 339 meters, and follow-up-6MWT test was 320 meters, the difference between pre and post result in both tests are insignificant and hence these results indicated the stability of the disease. Also, only 16.6% experienced drug adverse effect; 10% due to gastro-intestinal symptoms, 2.2% weight loss and 4.4% due to Photo-sensitivity. Conclusion: Pirfenidone stabilized IPF patients, as there was no decline in lung functions test and exercise capacity was preserved as demonstrated by the 6MWT. Perifenidone was well tolerated with acceptable safety profile.

Highlights

Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease, the disease carries high mortality and morbidity and if left untreated it may end up with respiratory failure and death within few years of diagnosis [1]
Clinical trials such as Capacity (004 & 006) suggested that Pirfenidone which is an anti-fibrotic and anti-inflammatory drug reduces the deterioration of patients with IPF in lung function and exercise capacity [5]. these result supported by the findings in ASCEND Clinical Trials, these trials showed that patients with IPF on Pirfenidone when compared to placebo group it reduces the disease progression as seen in lung function, exercise capacity and progression free survival, this study showed good safety profile of Pirfenidone [6]
The efficacy of the drug was assessed by Forced vital capacity (FVC); one parameter of lung function test; by comparing the result of Forced Vital Capacity (FVC) before starting Pirfenidone (Pre-FVC) with the last test done for the patient (Follow up-FVC), the patient assessed by six mint walk test (6MWT) as Pre-6-minutes’ walk test (6MWT) and Follow up-6MWT

Summary

Introduction

Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease, the disease carries high mortality and morbidity and if left untreated it may end up with respiratory failure and death within few years of diagnosis [1]. IPF is worse than many cancers as the the median survival from the time of diagnosis is 3 years and in the US 40,000 patients die because of IPF every year [4] Clinical trials such as Capacity (004 & 006) suggested that Pirfenidone which is an anti-fibrotic and anti-inflammatory drug reduces the deterioration of patients with IPF in lung function and exercise capacity [5]. These result supported by the findings in ASCEND Clinical Trials, these trials showed that patients with IPF on Pirfenidone when compared to placebo group it reduces the disease progression as seen in lung function, exercise capacity and progression free survival, this study showed good safety profile of Pirfenidone [6] Because of these encouraging results, pirfenidone is approved for the treatment of IPF in many countries.

Methodology

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Discussion