TY - JOUR T1 - Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00637-2022 SP - 00637-2022 AU - Eyjolfur Gudmundsson AU - An Zhao AU - Nesrin Mogulkoc AU - Frouke van Beek AU - Tinne Goos AU - Christopher J. Brereton AU - Marcel Veltkamp AU - Robert Chapman AU - Hendrik W. van Es AU - Helen Garthwaite AU - Bahareh Gholipour AU - Melissa Heightman AU - Arjun Nair AU - Katarina Pontoppidan AU - Recep Savas AU - Asia Ahmed AU - Marie Vermant AU - Omer Unat AU - Alex Procter AU - Laurens De Sadeleer AU - Emma Denneny AU - Timothy Wallis AU - Mark Duncan AU - Magali Taylor AU - Stijn Verleden AU - Sam M. Janes AU - Daniel C. Alexander AU - Athol U. Wells AU - Joanna Porter AU - Mark G Jones AU - Iain Stewart AU - Coline H.M. van Moorsel AU - Wim Wuyts AU - Joseph Jacob Y1 - 2023/01/01 UR - http://openres.ersjournals.com/content/early/2023/01/26/23120541.00637-2022.abstract N2 - Background Computer quantification of baseline computed tomography (CT) radiologic 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 radiologic 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, gender, smoking history, baseline emphysema presence, antifibrotic use and diffusion capacity for carbon monoxide. Mortality analyses further adjusted for baseline presence of clinically important PPFE-like lesions and ILD change.Findings 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 (HR=1.25, 95% CI 1.16–1.34, p<0.0001) and the FHP cohort (HR=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.FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.Conflict of interest: JJ reports fees from Boehringer Ingelheim, Roche, NHSX, Takeda and GlaxoSmithKline unrelated to the submitted work. JJ was supported by Wellcome Trust Clinical Research Career Development Fellowship 209553/Z/17/Z and the NIHR Biomedical Research Centre at University College London.Conflict of interest: SMJ reports fees from Astra-Zeneca, Bard1 Bioscience, Achilles Therapeutics, and Jansen unrelated to the submitted work. SMJ received assistance for travel to meetings from Astra Zeneca to American Thoracic Conference 2018 and from Takeda to World Conference Lung Cancer 2019 and is the Investigator Lead on grants from GRAIL Inc, GlaxoSmithKline plc and Owlstone.Conflict of interest: AUW personal fees and non-financial support from Boehringer Ingelheim, Bayer and Roche Pharmaceuticals; and personal fees from Blade, outside of the submitted work.Conflict of interest: EG has nothing to disclose.Conflict of interest: AZ has nothing to disclose.Conflict of interest: NM has nothing to disclose.Conflict of interest: IS has nothing to disclose.Conflict of interest: MGJ has nothing to disclose.Conflict of interest: CvM has nothing to disclose.Conflict of interest: RS has nothing to disclose.Conflict of interest: CJB has nothing to disclose.Conflict of interest: HWvE has nothing to disclose.Conflict of interest: OU has nothing to disclose.Conflict of interest: KP has nothing to disclose.Conflict of interest: FvB has nothing to disclose.Conflict of interest: TW has nothing to disclose.Conflict of interest: MV has nothing to disclose.Conflict of interest: BG has nothing to disclose.Conflict of interest: AN has nothing to disclose.Conflict of interest: DA has nothing to disclose.Conflict of interest: TG has nothing to disclose.Conflict of interest: RC has nothing to disclose.Conflict of interest: HG has nothing to disclose.Conflict of interest: MH has nothing to disclose.Conflict of interest: AA has nothing to disclose.Conflict of interest: AP has nothing to disclose.Conflict of interest: LDS has nothing to disclose.Conflict of interest: ED has nothing to disclose.Conflict of interest: MD has nothing to disclose.Conflict of interest: MT has nothing to disclose.Conflict of interest: SV has nothing to disclose.Conflict of interest: JP has nothing to disclose.Conflict of interest: WW has nothing to disclose. 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