ERJ Open Research (Mar 2023)

Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis

  • Eyjolfur Gudmundsson,
  • An Zhao,
  • Nesrin Mogulkoc,
  • Frouke van Beek,
  • Tinne Goos,
  • Christopher J. Brereton,
  • Marcel Veltkamp,
  • Robert Chapman,
  • Hendrik W. van Es,
  • Helen Garthwaite,
  • Bahareh Gholipour,
  • Melissa Heightman,
  • Arjun Nair,
  • Katarina Pontoppidan,
  • Recep Savas,
  • Asia Ahmed,
  • Marie Vermant,
  • Omer Unat,
  • Alex Procter,
  • Laurens De Sadeleer,
  • Emma Denneny,
  • Timothy Wallis,
  • Mark Duncan,
  • Magali Taylor,
  • Stijn Verleden,
  • Sam M. Janes,
  • Daniel C. Alexander,
  • Athol U. Wells,
  • Joanna Porter,
  • Mark G. Jones,
  • Iain Stewart,
  • Coline H.M. van Moorsel,
  • Wim Wuyts,
  • Joseph Jacob

DOI
https://doi.org/10.1183/23120541.00637-2022
Journal volume & issue
Vol. 9, no. 2

Abstract

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Background Computer quantification of baseline computed tomography (CT) radiological pleuroparenchymal fibroelastosis (PPFE) associates with mortality in idiopathic pulmonary fibrosis (IPF). We examined mortality associations of longitudinal change in computer-quantified PPFE-like lesions in IPF and fibrotic hypersensitivity pneumonitis (FHP). Methods Two CT scans 6–36 months apart were retrospectively examined in one IPF (n=414) and one FHP population (n=98). Annualised change in computerised upper-zone pleural surface area comprising radiological PPFE-like lesions (Δ-PPFE) was calculated. Δ-PPFE >1.25% defined progressive PPFE above scan noise. Mixed-effects models evaluated Δ-PPFE against change in visual CT interstitial lung disease (ILD) extent and annualised forced vital capacity (FVC) decline. Multivariable models were adjusted for age, sex, smoking history, baseline emphysema presence, antifibrotic use and diffusion capacity of the lung for carbon monoxide. Mortality analyses further adjusted for baseline presence of clinically important PPFE-like lesions and ILD change. Results Δ-PPFE associated weakly with ILD and FVC change. 22–26% of IPF and FHP cohorts demonstrated progressive PPFE-like lesions which independently associated with mortality in the IPF cohort (hazard ratio 1.25, 95% CI 1.16–1.34, p<0.0001) and the FHP cohort (hazard ratio 1.16, 95% CI 1.00–1.35, p=0.045). Interpretation Progression of PPFE-like lesions independently associates with mortality in IPF and FHP but does not associate strongly with measures of fibrosis progression.