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Abstract
Pirfenidone is an antifibrotic compound able to slow down disease progression in patients with idiopathic pulmonary fibrosis (IPF). To investigate the safety and efficacy of pirfenidone in patients with IPF in a real-life setting. This was a multicenter, retrospective, real-life, observational study for patients with IPF receiving pirfenidone. We identified 92 patients with IPF receiving pirfenidone. Eighty patients (70 males and 10 females, mean age ± SD: 68.1 + 7.5, mean %FVC ± SD = 74.9 ± 17.2, mean %DLCO ± SD = 48.1 ± 16.9) were included in the analysis. Skin-related (25%) and gastrointestinal (17.5%) adverse events were the most common and led to drug discontinuation in 22.5% of cases. The majority (87%) of patients experienced side effects during the first 6 months of treatment. At 36 months, changes in %FVC and %DLCO were -9.25 ± 16.34 and -9.26 ± 15.26, respectively. At 6, 12, and 24 months after treatment initiation (n = 80, 60, and 26), 18, 15, and 5 patients (22.5, 25, and 19.2%) experienced significant (>10%) and 11, 3, and 3 patients (13.8, 5, and 11.5%) experienced marginal (5-10%) %FVC improvement; and 13, 6, and 1 patient (16.2, 10, and 3.9%) experienced marginal (-5 to -10%) and 20, 21, and 8 patients (25, 35, and 30.8%) experienced significant decline (<-10%) in %FVCpred. Median survival was 851 days, and 41 patients died during the study period. Pirfenidone demonstrated an acceptable safety and therapeutic profile in patients with IPF on a longitudinal basis. Prospective observational registries are urgently needed to provide a real-world view of outcomes of pirfenidone in clinical practice.
Highlights
Idiopathic pulmonary fibrosis (IPF) represents a chronic, debilitating lung disease of unknown origin, characterized by irreversible loss of lung function due to lung scarring [1]
We aimed to report for the first time the longitudinal safety and efficacy outcomes of pirfenidone in patients with IPF derived from multiple clinical centers in Greece
Eleven patients (13.8%) underwent video-assisted thoracoscopic surgery (VATS) for lung biopsy which was consistent with UIP pattern in all cases
Summary
Idiopathic pulmonary fibrosis (IPF) represents a chronic, debilitating lung disease of unknown origin, characterized by irreversible loss of lung function due to lung scarring [1]. Four years ago FDA approved two novel anti-fibrotic compounds, pirfenidone and nintedanib, which are able to reduce disease progression in large multicenter clinical trials with IPF patients [9]. It has been demonstrated to slow down functional deterioration and reduces the risk of death by 48% at 1 year in a prespecified pooled analysis including data from three independent cohorts of patients with IPF [18, 19]. Pirfenidone is an antifibrotic compound able to slow down disease progression in patients with idiopathic pulmonary fibrosis (IPF)
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