Idiopathic Pulmonary Fibrosis in the United Kingdom: Analysis of the British Thoracic Society Electronic Registry between 2013 and 2019
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.