PT - JOURNAL ARTICLE AU - Martin J. Kohlhaeufl AU - Hermann Ingerl AU - Margit Bachnik TI - Pirfenidone in idiopathic pulmonary fibrosis - description of a German cohort DP - 2014 Sep 01 TA - European Respiratory Journal PG - P3749 VI - 44 IP - Suppl 58 4099 - http://erj.ersjournals.com/content/44/Suppl_58/P3749.short 4100 - http://erj.ersjournals.com/content/44/Suppl_58/P3749.full SO - Eur Respir J2014 Sep 01; 44 AB - Idiopathic Pulmonary Fibrosis (IPF) is a progressive and invariably deadly disease with a prognosis similar to that of many common malignancies. Until recently, there was no drug specifically licensed for the treatment of IPF. Pirfenidone was approved in the EU and Canada for the treatment of adults with mild to moderate IPF in 2011. Pirfenidone significantly reduces disease progression as compared to placebo (Noble PW, et al. Lancet 2011;377:1760-1769), but side effects may lead to compliance problems and/or discontinuation of treatment. In spite of this, 80% of patients stay on treatment, and side effects are usually reversible, treatable, and decrease over time (Valeyre D, et al. Eur Resp J 2012; 40 (Suppl 56):563s).This is a prospective observational single-centre cohort study at a pneumological referral centre. The target is to collect real world clinical data of Pirfenidone in 31 IPF patients for a period of about 2 years. The patients are being examined at regular intervals, and according to routine clinical practice the examinations include lung function tests, radiological imaging and blood tests, as well as assessment of possible side effects. At baseline, the mean age was 69 years, with 28 male and 3 female patients; 20 of the patients were former smokers, 11 were non-smokers, 21 needed supplemental oxygen, and HRCT data were available for 24; mean FEV1.0 was 2,3 L, mean FVC was 2.7 L; the median time since IPF diagnosis was 2 years.The data presented cover a period from May 2012 (first patient in) until mid-2014. So far, Pirfenidone showed a favourable benefit/risk ratio in the majority of patients in this cohort.