TY - JOUR T1 - Idiopathic Pulmonary Fibrosis in the United Kingdom: Analysis of the British Thoracic Society Electronic Registry between 2013 and 2019 JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00187-2020 SP - 00187-2020 AU - LG Spencer AU - M Loughenbury AU - C Chaudhuri AU - M Spiteri AU - H Parfrey A2 - , Y1 - 2020/01/01 UR - http://openres.ersjournals.com/content/early/2020/09/17/23120541.00187-2020.abstract N2 - Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive and terminal interstitial lung disease (ILD) with a median survival of 3–5 years. The British Thoracic Society (BTS) established the UK IPF Registry in 2013 as a platform to collect data on clinical characteristics, treatments and outcomes for this cohort in the UK.Between 1st January 2013 and 31st October 2019, 2474 cases were registered. Most patients were male (79%) with a mean (sd) age of 74±8.3 and 66% were ex-smokers. Over time we observed an increase in the number of patients aged over 70. However, we have not seen a trend towards earlier presentation as symptoms of breathless and/or cough were present for more than 12 months in 63% of the cohort. At presentation, mean (sd) percent predicted FVC was 78.2±18.3, median 76.2 (IQR 65.888.2) and TLco 48.4±16.0, median 47.5 (IQR 37.3, 57.4). Most cases were discussed at an ILD multi-disciplinary meeting, with an increase over this time in the number of cases reported as having possible UIP pattern on HRCT thorax. We noted a reduction in the number of patients undergoing surgical lung biopsy or bronchoalveolar lavage. Although more patients were prescribed anti-fibrotic therapies from 2013 to 2019, 43% were ineligible for treatment based upon NICE prescribing criteria. Hypertension, ischaemic heart disease, diabetes mellitus and gastro-oesophageal reflux were the most common co-morbidities.In conclusion, we have presented baseline demographics as well as diagnostic and treatment strategies from the largest single-country IPF registry, reflecting changes in UK practices over this period.FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.Conflict of interest: L.G. Spencer reports a small, one-off start grant from HQIP in 2013, and small, one-off grants for software from Boehringer Ingelheim and InterMune in 2014, during the conduct of the study; and in the last 12 months has given one lecture paid for by Roche, facilitated one 2-day leadership course paid for by Boehringer Ingelheim, and received travel support and a registration fee to attend ERS Congress 2019 from Roche.Conflict of interest: M. Loughenbury reports a small, one-off start grant from HQIP in 2013, and small, one-off grants for software from Boehringer Ingelheim and InterMune in 2014, during the conduct of the study.Conflict of interest: C. Chaudhuri reports a small, one-off starter grant from HQIP in 2013, and small, one-off grants for software from Boehringer Ingelheim and InterMune in 2014, during the conduct of the study; and in the last 12 months: Roche funding for attending ATS 2019, participation in advisory boards, presenting at UK Advances in IPF meeting, presenting at Roche European AIR meeting and presenting at international meetings, and Boehringer Ingelheim funding for attending ERS 2019, participation in advisory boards and a video for a clinical trial.Conflict of interest: M. Spiteri reports a small, one-off start grant from HQIP in 2013, and small, one-off grants for software from Boehringer Ingelheim and InterMune in 2014, during the conduct of the study.Conflict of interest: H. Parfrey reports a small, one-off start grant from HQIP in 2013, and small, one-off grants for software from Boehringer Ingelheim and InterMune in 2014, during the conduct of the study; an educational grant and costs for conference attendance from Boehringer Ingelheim, and an educational grant, lecture fees and costs for conference attendance from Roche, outside the submitted work; and she is founding trustee for Action for Pulmonary Fibrosis. ER -