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  • P163 A multicentre retrospective cohort comparison of aetiology and survival in patients with chronic hypersensitivity pneumonitis versus idiopathic pulmonary fibrosis

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Updates on treatment, prognosis and outcomes in ILD
P163 A multicentre retrospective cohort comparison of aetiology and survival in patients with chronic hypersensitivity pneumonitis versus idiopathic pulmonary fibrosis
  1. SL Barratt1,
  2. HI Adamali1,
  3. A Creamer1,
  4. A Duckworth2,
  5. J Fallon3,
  6. MA Gibbons4,
  7. B Gooptu5,
  8. T Nancarrow2,
  9. J Pepperell3,
  10. AS Spiers4,
  11. RA Stone3,
  12. RL Wollerton4,
  13. FA Woodhead6,
  14. CJ Scotton2
  1. 1Bristol Interstitial Lung Disease Service, North Bristol NHS Trust, Bristol, UK
  2. 2University of Exeter Medical School, Exeter, UK
  3. 3Somerset Lung Centre, Musgrove Park Hospital, Taunton, UK
  4. 4Respiratory Department Royal Devon and Exeter NHS Foundation Trust Exeter, Exeter, UK
  5. 5Leicester Institute of Structural and Chemical Biology/NIHR Leicester BRC – Respiratory, University of Leicester, Leicester, UK
  6. 6Institute for Lung Health, Glenfield Hospital, University Hospitals of Leicester NHS Trust, Leicester, UK

Abstract

Introduction Hypersensitivity pneumonitis is a complex syndrome believed to result from repeated inhalation and sensitisation to a variety of antigenic stimuli. The majority of cases are classified as chronic and may result in fibrotic progression which can be hard to distinguish from idiopathic pulmonary fibrosis (IPF). Precise aetiology and optimal management strategies are less well defined than for IPF, although clinical trials exploring efficacy of pirfenidone (NCT02496182) and nintedanib (NCT02999178) are in progress.

Objectives To establish a large, UK-based, multicentre, retrospective cohort of patients with cHP to facilitate detailed investigation of patient demographics, management and prognosis.

Methods Multicentre evaluation of clinical data for patients presenting to the interstitial lung disease clinics at the North Bristol NHS Trust, University Hospitals of Leicester NHS Trust, Taunton and Somerset NHS Foundation Trust and the Royal Devon & Exeter NHS Foundation Trust between 2005 and 2018. Basic demographics and survival were compared using chi-square test with Yates correction (gender), Mann-Whitney U-test (age at presentation) and Kaplan-Meier Survival analysis in Graphpad Prism.

Results In total, 397 patients with a diagnosis of cHP were identified, compared with 1068 patients with IPF. There was a significant difference in age at diagnosis between cHP and IPF (median 72.0 vs 75.6, p<0.0001), and a highly significant difference in gender distribution, with a greater proportion of women presenting with cHP than men (237 female vs 160 male, p<0.001), in stark contrast to IPF (246 female vs 822 male). cHP was associated with a better prognosis than IPF (p<0.05), with a median survival of 7.1 years following diagnosis compared with 3.0 years for IPF.

Conclusions Despite challenges in diagnosing cHP (as demonstrated by Walsh et al 1 in their review of UK MDT agreement where HP has only a fair kappa score of 0.29) and in line with published literature, our cHP cohort has a female preponderance and is associated with better survival than IPF. This cohort will provide a crucial resource for more detailed analysis of patient data (e.g. spirometry and response to therapy).

Reference

  1. Walsh SL, et al. Lancet Respir Med2016;4:557–65.

https://doi.org/10.1136/thorax-2018-212555.321

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