TY - JOUR T1 - Pirfenidone treatment in idiopathic pulmonary fibrosis: An Italian case series JF - European Respiratory Journal JO - Eur Respir J VL - 42 IS - Suppl 57 SP - P3370 AU - Claudia Ravaglia AU - Carlo Gurioli AU - Micaela Romagnoli AU - GianLuca Casoni AU - Sara Tomassetti AU - Christian Gurioli AU - Venerino Poletti Y1 - 2013/09/01 UR - http://erj.ersjournals.com/content/42/Suppl_57/P3370.abstract N2 - Background and objective: Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic interstitial lung disease with an estimated median survival of only 2-3 years from initial diagnosis. To date, Pirfenidone is the only pharmacological agent that has been approved in the European Union for the treatment of IPF. We report our experience on efficacy of pirfenidone in IPF in our centre.Patients and methods: we studied all the patients with a diagnosis of IPF who have been treated with Pirfenidone over the period from September 2008 to February 2013 in our centre; follow-up time was calculated from the starting point of pirfenidone to the last patient visit or to the interruption of the drug.Results: 81 patients with IPF were treated with pirfenidone but only 68 were included in the study as 13 patients had discontinued the drug in the first thrre months of treatment because of side effects. Mean follow-up time was 43,48 weeks [range 12 - 219], mean age was 69 years [range 41 - 81], 58 were male (85%) and 10 were female (15%). Mean FVC at the starting point of pirfenidone was 70,75% and DLCO was 42,82%. At the end of the follow-up period mean FVC was 69,26% and DLCO was 41,39%; 40 patients (58,8 %) were stable or have significantly improved after treatment while 20 patients (29,4 %) have significantly worsened after treatment. Patients who got worse were all smokers but 2 (90%).Conclusion: Pirfenidone was generally well tolerated and the course of the disease was stable during treatment with pirfenidone in the majority of patients. Our results are in line with the previous published safety and efficacy data on pirfenidone as treatment for IPF. ER -