ERJ Open Research (Jan 2021)

Idiopathic pulmonary fibrosis in the UK: analysis of the British Thoracic Society electronic registry between 2013 and 2019

  • Lisa G. Spencer,
  • Maria Loughenbury,
  • Nazia Chaudhuri,
  • Monica Spiteri,
  • Helen Parfrey,
  • BTS ILD Registry,
  • Christine Fiddler,
  • Gareth Walters,
  • Sherwood Burge,
  • Melanie Caswell,
  • Simon Hart,
  • Mark Major,
  • David Adeboyeku,
  • Ananthakrishnan Raghuram,
  • Ling-Pei Ho,
  • Arvind Rajasekaran,
  • Aravind Ponnuswamy,
  • Stephen Cowie,
  • Mamoun Ibrahim,
  • Christine O'Brien,
  • Jane Scullion,
  • Andrew White,
  • Henry Steer,
  • Dimitrina Petkova,
  • Surinder Birring,
  • Boris Lams,
  • Robina Coker,
  • Robert Buttery,
  • Hannah Fletcher,
  • John Hutchinson,
  • Emad Hilal,
  • Janet Fallon,
  • Ahmed Fahim,
  • Andrew Wilson,
  • Georgina Hands,
  • Alison Moody,
  • Stephen Bianchi,
  • Bhagyashree Jayaraman,
  • Arnab Datta,
  • William Chang,
  • Jonathan Naylor,
  • K. Suresh Babu,
  • Robert Allcock,
  • Philip Molyneaux,
  • Srividya Narayan,
  • Michael Gibbons,
  • Katie Ward,
  • Timothy Gatheral,
  • Claire Squires,
  • Ian Forrest,
  • Mazhar Chaudri,
  • Salman Ghani,
  • Katherine Spinks,
  • Huzaifa Adamali,
  • Paul Beirne,
  • Melissa Wickremasinghe,
  • Joanna Porter,
  • Beatriz Lara,
  • Graham Miller,
  • Sarah Haney,
  • Laura MacKay,
  • Steve O'Hickey,
  • Owen Dempsey,
  • Matthew Embley,
  • George Chalmers,
  • Sarah Davies,
  • Ben Hope-Gill,
  • Neil McAndrew,
  • Paul Minnis,
  • Karol Henry

DOI
https://doi.org/10.1183/23120541.00187-2020
Journal volume & issue
Vol. 7, no. 1

Abstract

Read online

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 1 January 2013 and 31 October 2019, 2474 cases were registered. Most patients were male (79%) with a mean±sd age of 74±8.3 years and 66% were ex-smokers. Over time we observed an increase in the number of patients aged over 70 years. However, we have not seen a trend towards earlier presentation as symptoms of breathless and/or cough were present for >12 months in 63% of the cohort. At presentation, mean±sd % predicted forced vital capacity (FVC) was 78.2±18.3%, median 76.2% (interquartile range (IQR) 22.4%) and transfer factor of the lung for carbon monoxide (TLCO) 48.4±16.0, median 47.5 (IQR 20.1). Most cases were discussed at an ILD multidisciplinary meeting, with an increase over this time in the number of cases reported as having possible usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (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 National Institute for Health and Care Excellence (NICE) prescribing criteria. Hypertension, ischaemic heart disease, diabetes mellitus and gastro-oesophageal reflux were the most common comorbidities. 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.