Ismail Hanta1, Aykut Cilli2, Can Sevinc3. 1. Department of Chest Diseases, Çukurova University, Adana, Turkey. ismailhnt@gmail.com. 2. Department of Chest Diseases, Akdeniz University, Antalya, Turkey. 3. Department of Chest Diseases, Dokuz Eylül University, İzmir, Turkey.
Abstract
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.
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.
Authors: Sebastian Majewski; Adam J Białas; Małgorzata Buchczyk; Paweł Gomółka; Katarzyna Górska; Hanna Jagielska-Len; Agnieszka Jarzemska; Ewa Jassem; Dariusz Jastrzębski; Aleksander Kania; Marek Koprowski; Rafał Krenke; Jan Kuś; Katarzyna Lewandowska; Magdalena M Martusewicz-Boros; Kazimierz Roszkowski-Śliż; Alicja Siemińska; Krzysztof Sładek; Małgorzata Sobiecka; Karolina Szewczyk; Małgorzata Tomczak; Witold Tomkowski; Elżbieta Wiatr; Dariusz Ziora; Beata Żołnowska; Wojciech J Piotrowski Journal: BMC Pulm Med Date: 2020-05-04 Impact factor: 3.317