TY - JOUR T1 - Pirfenidone in idiopathic pulmonary fibrosis - Real life experience from a german tertiary referral centre for interstitial lung diseases JF - European Respiratory Journal JO - Eur Respir J VL - 42 IS - Suppl 57 SP - P2335 AU - Ute Oltmanns AU - Nicolas Kahn AU - Heinrich Wenz AU - Claus-Peter Heussel AU - Michael Puderbach AU - Svenja Ehlers-Tenenbaum AU - Arne Warth AU - Felix Herth AU - Philip Schnabel AU - Michael Kreuter Y1 - 2013/09/01 UR - http://erj.ersjournals.com/content/42/Suppl_57/P2335.abstract N2 - Introduction: Pirfenidone is a newly licensed antifibrotic drug that might slow down disease progression and increase progression free survival in idiopathic pulmonary fibrosis (IPF). However, side effects may offset treatment benefits and patient compliance. Recent course of disease, side effects and compliance were analysed in a tertiary ILD centre observation.Methods: 60 patients suffering from mild-moderate IPF according to ATS guidelines started pirfenidone therapy after consensual multidisciplinary ILD Board decision. Pulmonary function tests and treatment side effects were recorded at each clinic visit. Data are presented as median (interquartile range 25-75%). Disease progression was defined as a reduction of vital capacity (VC) ≥10% or diffusion capacity (DLCO) ≥15%.Results: Patient age was 70 (63-74) years, with a VC of 68 (58-80)% and a DLCO of 38 (30-52)% of predicted normal. Follow-up time on treatment was 210 (120-383) days. 7 patients (12%) showed disease progression. Overall VC and DLCO changed by 0 (-6 to +2)% and 2 (-3 to +9)%, respectively. 38 patients (66%) reported treatment-related side effects leading to drug discontinuation in 8 patients (14%). In order of frequency, reported side effects were: nausea, anorexia, fatigue, skin reactions, dizziness and myalgias. Other reasons for treatment termination were death (n=4), incompliance (n=3) and lung transplantation (n=1).Discussion: The majority of IPF patients treated with pirfenidone showed stable disease. Drug-related side effects are common but mostly manageable with supportive care, however, treatment discontinuation was required in 1 out of 7. ER -