The Effectiveness, Safety, and Tolerability of Pirfenidone in Idiopathic Pulmonary Fibrosis: A Retrospective Study.

Introduction: In this study we aimed to investigate the effectiveness and safety profile of pirfenidone for the treatment of idiopathic pulmonary fibrosis (IPF) in a real-life setting.

Methods: Clinical records of patients diagnosed with mild-to-moderate IPF and receiving pirfenidone treatment across three centers in Turkey between January and September 2017 were retrospectively collected. Pulmonary function measurements, including percentage of forced vital capacity (FVC%) and percentage of diffusion capacity (DLCO%) were analyzed in patients who received pirfenidone treatment for at least 6 months. Decline in lung function, defined as an absolute decline of at least 10% in FVC from baseline, or death at 6 months was also analyzed. Safety data were included for all follow-up visits.

Results: In the pooled cohort (n = 60), patients were mostly men (73.4%) and current or former smokers (61.7%). Average baseline FVC% and DLCO% were 68.4% and 48.7%, respectively. Forty-seven patients (78.3%) had a high-resolution computed tomography scan with a definite interstitial pneumonia (UIP) pattern, and 18 patients (30%) had a surgically proven UIP pattern. Forty-six (76.7%) patients with IPF remained stable and 14 (23.3%) patients had progressed according to decline in FVC of at least 10% during the therapy course. After 6 months of therapy, cough decreased in 58.3% of patients. At least one side effect due to therapy was encountered in 33 (55.0%) IPF patients. Dyspepsia (36.4%), nausea (27.3%), and rash/photosensitivity (24.2%) were the most frequent side effects in our cohort. Sixteen patients (26.7%) needed dose adjustment, one patient (1.7%) discontinued therapy, and one patient (1.7%) died in the study period.

Conclusions: This study shows that pirfenidone seems to be an effective treatment for IPF and also had tolerable and relatively acceptable side effects.

Funding: Roche.