TY - JOUR T1 - Real World experiences: Pirfenidone is well tolerated and reduces decline in FVC and TLCO at 9 months in IPF JF - European Respiratory Journal JO - Eur Respir J VL - 42 IS - Suppl 57 SP - P2333 AU - Nazia Chaudhuri AU - Annette Duck AU - Jayne Holme AU - Rebecca Frank AU - Colm Leonard Y1 - 2013/09/01 UR - http://erj.ersjournals.com/content/42/Suppl_57/P2333.abstract N2 - INTRODUCTION: IPF has a 2-5 year mortality from diagnosis. Pirfenidone is the only licenced drug in Europe for IPF. Here we demonstrate our experiences in the UK with Pirfenidone on the named patient programme (NPP).METHODS: This was a single centre observational study. We retrospectively analysed the data from 40 patients treated with Pirfenidone from September 2011 to January 2012.RESULTS: Mean age was 65.8 years (range 48-80) with 70% (28/40) males. The average duration of treatment was 42.3 weeks (range 11-74). During this period 9 (22%) patients died due to progression of their IPF. 21/40 (53%) patients experienced gastrointestinal adverse effects. Of these 7 (34%) were self-limiting. 4 (19%) resolved after a dose reduction, in 3 (14%) patients Pirfenidone was restarted after the adverse effect settled and in 7 (33%; 17% total) Pirfenidone was discontinued.Nine months before and after Pirfenidone commencement we observed a reduction in the decline of FVC and TLCO with a difference in gradients pre and post Pirfenidone of -1.02 vs 0.07 and -2.37 vs 0.07 respectively.CONCLUSION: Pirfenidone is well tolerated. Adverse effects are predominantly gastrointestinal, self-limiting and occur within 8 weeks of the start of treatment. Although our numbers are small we demonstrate a reduction in the decline of FVC and TLCO at 9 months. Further longitudinal follow-up is required. ER -