PMID- 33532476
OWN - NLM
STAT- PubMed-not-MEDLINE
LR  - 20210206
IS  - 2312-0541 (Print)
IS  - 2312-0541 (Electronic)
IS  - 2312-0541 (Linking)
VI  - 7
IP  - 1
DP  - 2021 Jan
TI  - Idiopathic pulmonary fibrosis in the UK: analysis of the British Thoracic Society 
      electronic registry between 2013 and 2019.
LID - 00187-2020 [pii]
LID - 10.1183/23120541.00187-2020 [doi]
AB  - 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 (T (LCO)) 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.
CI  - Copyright (c)ERS 2021.
FAU - Spencer, Lisa G
AU  - Spencer LG
AUID- ORCID: 0000-0003-3558-992X
AD  - Aintree University Hospital, Liverpool, UK.
FAU - Loughenbury, Maria
AU  - Loughenbury M
AD  - British Thoracic Society, London, UK.
FAU - Chaudhuri, Nazia
AU  - Chaudhuri N
AD  - Wythenshawe Hospital, Manchester, UK.
FAU - Spiteri, Monica
AU  - Spiteri M
AD  - University Hospital of North Midlands, Stoke on Trent, UK.
FAU - Parfrey, Helen
AU  - Parfrey H
AUID- ORCID: 0000-0002-4515-5634
AD  - Royal Papworth Hospital, Cambridge, UK.
LA  - eng
PT  - Journal Article
DEP - 20210125
PL  - England
TA  - ERJ Open Res
JT  - ERJ open research
JID - 101671641
PMC - PMC7836645
COIS- 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: N. 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.
EDAT- 2021/02/04 06:00
MHDA- 2021/02/04 06:01
PMCR- 2021/01/25
CRDT- 2021/02/03 06:00
PHST- 2020/04/15 00:00 [received]
PHST- 2020/09/13 00:00 [accepted]
PHST- 2021/02/03 06:00 [entrez]
PHST- 2021/02/04 06:00 [pubmed]
PHST- 2021/02/04 06:01 [medline]
PHST- 2021/01/25 00:00 [pmc-release]
AID - 00187-2020 [pii]
AID - 10.1183/23120541.00187-2020 [doi]
PST - epublish
SO  - ERJ Open Res. 2021 Jan 25;7(1):00187-2020. doi: 10.1183/23120541.00187-2020. 
      eCollection 2021 Jan.