Abstract
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.
Footnotes
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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.
This is a PDF-only article. Please click on the PDF link above to read it.
- Received November 21, 2022.
- Accepted December 1, 2022.
- Copyright ©The authors 2023.
This version is distributed under the terms of the Creative Commons Attribution Licence 4.0.
