TY - JOUR T1 - Pirfenidone in patients with advanced idiopathic pulmonary fibrosis: First experience JF - European Respiratory Journal JO - Eur Respir J DO - 10.1183/13993003.congress-2016.PA787 VL - 48 IS - suppl 60 SP - PA787 AU - Filip Hoet AU - Jonas Yzerbyt AU - Wim A. Wuyts Y1 - 2016/09/01 UR - http://erj.ersjournals.com/content/48/suppl_60/PA787.abstract N2 - Introduction: Recently Pirfenidone and Nintedanib were recommended for use in patients with IPF by the ATS/ERS/JRS/ALAT guidelines [Raghu G, et al. Am J Respir Crit Care Med 2015; 192: e3 – e19]. It is not known whether patients with advanced pulmonary function impairment might also benefit from these therapies.Aims: To evaluate the efficacy and safety of Pirfenidone in patients with more advanced pulmonary function impairment, defined as FVC ≤ 50% and/or DLco ≤ 35 %.Methods: We retrospectively analysed pulmonary function data of all patients who started treatment with Pirfenidone in our centre before September 2014, and who had developed more severe pulmonary function impairment (FVC ≤ 50% and/or DLco ≤ 35 %). The evolution of the pulmonary function tests during the 6 months before and after initiation of Pirfenidone was compared using a single-sided pared T-test.Results: 23 patients were included in the study. 4 patients had stopped therapy and 3 patients had died before 6 months on treatment with Pirfenidone. The remaining 16 patients had a significantly reduced decline in FVC (+ 38,8 ml (SD 204,9) vs - 187,5 ml (SD 284,4) or + 1,4 % (SD 5,7) vs - 4,8 % (SD 7,3)) and DLco (- 0,9 % (SD 5,0) vs - 11,3 % (SD 10,8)) in the first 6 months on therapy as compared to the last 6 months before initiation of Pirfenidone. There was also an acceptable tolerance profile.Conclusions: This analysis suggests that also patients with more severely altered pulmonary function impairment could still benefit from treatment with Pirfenidone, with an acceptable side-effect profile. ER -