RT Journal Article
SR Electronic
T1 Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis
JF ERJ Open Research
JO erjor
FD European Respiratory Society
SP 00637-2022
DO 10.1183/23120541.00637-2022
VO 9
IS 2
A1 Eyjolfur Gudmundsson
A1 An Zhao
A1 Nesrin Mogulkoc
A1 Frouke van Beek
A1 Tinne Goos
A1 Christopher J. Brereton
A1 Marcel Veltkamp
A1 Robert Chapman
A1 Hendrik W. van Es
A1 Helen Garthwaite
A1 Bahareh Gholipour
A1 Melissa Heightman
A1 Arjun Nair
A1 Katarina Pontoppidan
A1 Recep Savas
A1 Asia Ahmed
A1 Marie Vermant
A1 Omer Unat
A1 Alex Procter
A1 Laurens De Sadeleer
A1 Emma Denneny
A1 Timothy Wallis
A1 Mark Duncan
A1 Magali Taylor
A1 Stijn Verleden
A1 Sam M. Janes
A1 Daniel C. Alexander
A1 Athol U. Wells
A1 Joanna Porter
A1 Mark G. Jones
A1 Iain Stewart
A1 Coline H.M. van Moorsel
A1 Wim Wuyts
A1 Joseph Jacob
YR 2023
UL http://openres.ersjournals.com/content/9/2/00637-2022.abstract
AB 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.Computerised pleuroparenchymal fibroelastosis (PPFE) progression was found to associate with mortality in idiopathic pulmonary fibrosis and fibrotic hypersensitivity pneumonitis but PPFE did not correlate strongly with measures of fibrosis progression https://bit.ly/3FKYQn7