Abstract
BackgroundPirfenidone 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.MethodsThis is a retrospective observational intention to treat study of 82 consecutive IPF patients (UHH cohort).ResultsWe 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.
Highlights
Pirfenidone is an antifibrotic compound approved for the treatment of idiopathic pulmonary fibrosis (IPF)
A pooled analysis of these trials and a meta-analysis including a Japanese phase 2 trial, SP2, and a Japanese phase 3 trial, SP3 confirmed that pirfenidone is associated with a reduced risk of death compared to placebo and the survival benefit of pirfenidone extended beyond 52 weeks and seemed to persist at 120 weeks [7]
The radiologic pattern of definite usual interstitial pneumonia (UIP) on high resolution computed tomography (HRCT) was seen in 47 patients (57.3%) and the pattern of possible UIP was seen in 35 (42.7%)
Summary
Pirfenidone is an antifibrotic compound approved for the treatment of idiopathic pulmonary fibrosis (IPF). Pirfenidone is an orally administered drug with antifibrotic, anti-inflammatory and antioxidant effects that was approved in Europe in 2011 and in USA in 2014 for the treatment of IPF [4]. Three multinational, randomised, placebo-controlled phase III trials showed that pirfenidone can reduce the rate of IPF progression by 50% on average in 1 year as judged by serial changes in forced vital capacity (FVC) [6]. These trials were not powered to explore the effect of pirfenidone on mortality. A pooled analysis of these trials and a meta-analysis including a Japanese phase 2 trial, SP2 (trial duration 9 months), and a Japanese phase 3 trial, SP3 (trial duration 52 weeks) confirmed that pirfenidone is associated with a reduced risk of death compared to placebo and the survival benefit of pirfenidone extended beyond 52 weeks and seemed to persist at 120 weeks [7]
Sign-in/Register to view highlights & summary 
Published Version (
Free)
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a call