Treatment of idiopathic pulmonary fibrosis (IPF) with Esbriet (pirfenidone) leads to stable lung function over two years, according to a real-world study. The data matches previous results from Phase 3 trials.
The research, “Functional decline over time in patients with IPF treated with pirfenidone: the PROOF registry,” was presented at the European Respiratory Society (ERS) International Congress 2018, held in Paris, France, Sept. 15-19.
Although the effectiveness and safety of IPF treatment with the anti-fibrotic drugs Esbriet (marketed by Genentech) or Ofev (nintedanib, marketed by Boehringer Ingelheim) is supported by data from Phase 3 trials, long-term data in real-world IPF populations are still lacking.
Aiming to address this gap, the PROOF registry was started in 2013 to track disease progression in a real-world population of patients with IPF. The registry includes adult patients with a diagnosis of probable or definite IPF from eight centers in Belgium and Luxembourg.
The study, sponsored by Roche, which owns Genentech, assessed changes in lung function over 24 months in IPF patients treated with Esbriet. Specifically, lung function was determined at the time of study inclusion (month 0) and at months 3, 6, 12 and 24, through percent predicted forced vital capacity (%FVC) and percent predicted diffusing capacity for carbon monoxide (%DLco), which is a test of the lungs’ capacity to transfer oxygen from the air sacs into the blood.
Results showed that 233 (84.1%) of 277 IPF patients in the PROOF registry received Esbriet for any duration of treatment. Mean duration of treatment was 20.1 months, with 87.1% of patients treated without interruption until discontinuation or end of follow-up.
Of the Esbriet-treated patients, 223 (95.7%) had a definite diagnosis of IPF, while 10 (4.3%) had a probable diagnosis.
Data of both %FVC and %DLco showed that lung function remained stable in patients treated with Esbriet.
Specifically, mean %FVC was 81.2% at month 0, 77.8% at month 3, 80.9% at month 6, 82.1% at month 12, and 78.3% at month 24. In turn, mean %DLco was 47.0%, 46.1%, 45.1%, 45.4% and 45.0% at months 0, 3, 6, 12 and 24, respectively.
The analyses also revealed a 10% or greater absolute %FVC decline compared to month 0 (baseline) in 11.1%, 16.6%, 19.1%, and 31.0% of patients at 3, 6, 12 and 24 months, respectively. In turn, 4.4%, 6.6%, 6.6% and 23.2% of patients experienced a 15% or greater absolute decline in %DLco vs. study start.
The mean time to achieve a 10% absolute decline in %FVC was 20.1 months, while 15% or greater absolute decline in %DLco was seen after a mean period of 23.4 months.
Based on the results, the team concluded: “pulmonary function remained largely stable during 24 months of follow-up in the majority of ever-treated patients who received pirfenidone [Esbriet] for any duration of treatment in PROOF.”
Among the study’s limitations, the researchers mentioned the relatively small group of patients from a limited geographic area. Missing data due to patients lost, to follow-up, or data not collected at all time points for all patients, were additional limitations.
Of note, the study was sponsored by Roche and two of the study’s authors are either in speakers bureau or received consulting and/or lecture fees from Roche and Boehringer Ingelheim.