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Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis

Title: Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis
Authors: Gudmundsson,Eyjolfur; Zhao,An; Mogulkoc,Nesrin; van Beek,Frouke; Goos,Tinne; Brereton,Christopher J.; Veltkamp, Marcel; Chapman,Robert; van Es,Hendrik W.; Garthwaite,Helen; Gholipour,Bahareh; Heightman,Melissa; Nair,Arjun; Pontoppidan,Katarina; Savas,Recep; Ahmed,Asia; Vermant,Marie; Unat,Omer; Procter,Alex; De Sadeleer,Laurens; Denneny,Emma; Wallis,Timothy; Duncan,Mark; Taylor,Magali; Verleden,Stijn; Janes,Sam M.; Alexander,Daniel C.; Wells,Athol U.; Porter,Joanna; Jones,Mark G.; Stewart,Iain; van Moorsel,Coline H.M.; Wuyts,Wim; Jacob,Joseph; Longziekten
Publication Year: 2023
Subject Terms: Pulmonary and Respiratory Medicine
Description: 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
Document Type: article in journal/newspaper
File Description: text/plain
Language: English
ISSN: 2312-0541
Relation: https://dspace.library.uu.nl/handle/1874/459054
Availability: https://dspace.library.uu.nl/handle/1874/459054
Rights: info:eu-repo/semantics/OpenAccess
Accession Number: edsbas.F6BB9CC4
Database: BASE