Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis.

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.

Informations de copyright

Copyright ©The authors 2023.

Déclaration de conflit d'intérêts

Conflict of interest: J. Jacob reports fees from Boehringer Ingelheim, Roche, NHSX, Takeda and GlaxoSmithKline unrelated to the submitted work. J. Jacob was supported by Wellcome Trust Clinical Research Career Development Fellowship 209553/Z/17/Z and the NIHR Biomedical Research Centre at University College London. S.M. Janes reports fees from AstraZeneca, Bard1 Bioscience, Achilles Therapeutics and Janssen unrelated to the submitted work. S.M. Janes received assistance for travel to meetings from AstraZeneca 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. A.U. Wells reports personal fees and nonfinancial support from Boehringer Ingelheim, Bayer and Roche Pharmaceuticals; and personal fees from Blade, outside of the submitted work. The remaining authors have nothing to disclose.