Background: Pirfenidone is an antifibrotic compound approved for the treatment of idiopathic pulmonary fibrosis (IPF). We present our real-world experience in terms of Pirfenidone's effect on mortality and adverse events profile outside the restrictions of a clinical trial. Methods: This is a retrospective observational intention to treat study of 82 consecutive IPF patients (UHH cohort). Results: We observed a high 3-years survival rate of 73% without excluding patients who discontinued treatment for different reasons. The survival was compared to the survival of an IPF cohort from a tertiary referral center (RBH cohort). After exclusion of severe cases (DLco< 30%), in unadjusted analysis, the survival in the UHH cohort was better than in the RBH cohort (HR:0.32, 95% CI: 0.19-0.53, p < 0.0001). After adjustment for age, gender and FVC, the survival remained higher in the UHH cohort (HR:0.28, 95% CI: 0.16-0.48, p < 0.0001). We observed a similar safety profile compared to previously published data and a lower rate of drug discontinuation due to photosensitivity reactions. Conclusion: Pirfenidone provides a survival benefit in a real-life IPF cohort compared to previously used medications. Counselling patients and proactively managing possible adverse effects can reduce the necessity to discontinue pirfenidone.
CITATION STYLE
Margaritopoulos, G. A., Trachalaki, A., Wells, A. U., Vasarmidi, E., Bibaki, E., Papastratigakis, G., … Antoniou, K. M. (2018). Pirfenidone improves survival in IPF: Results from a real-life study. BMC Pulmonary Medicine, 18(1). https://doi.org/10.1186/s12890-018-0736-z
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