PT - JOURNAL ARTICLE 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 TI - Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis AID - 10.1183/23120541.00637-2022 DP - 2023 Mar 01 TA - ERJ Open Research PG - 00637-2022 VI - 9 IP - 2 4099 - http://openres.ersjournals.com/content/9/2/00637-2022.short 4100 - http://openres.ersjournals.com/content/9/2/00637-2022.full SO - erjor2023 Mar 01; 9 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