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Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis

Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis
Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis
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 (∆-PPFE) was calculated. ∆-PPFE >1.25% defined progressive PPFE above scan noise. Mixed-effects models evaluated ∆-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: ∆-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 correlate strongly with measures of fibrosis progression.
2312-0541
Gudmundsson, Eyjolfur
9c6805d8-0f33-4872-a99f-44f0fbf19bf7
Zhao, An
1c74dd2e-8108-46b7-926f-1810e49bcd6b
Mogulkoc, Nesrin
433ea942-9ac8-4c78-86a3-f1d003043852
Wells, Athol
9e166381-f8f5-4007-968f-380f3663752a
Jones, Mark
a6fd492e-058e-4e84-a486-34c6035429c1
van Moorsel, Coline
bd92cbbd-c977-4eb6-a09a-fa273d8fc368
Porter, Joanna
83ce24e7-4f91-4ce5-9702-fa6a86074c6f
Wuyts, Wim
10be264e-5be4-42f4-9e90-43fa6474f82a
Stewart, Iain
4881bb01-497f-4ff9-8f23-36b513e944ee
Jacob, Joseph
de510fa4-f11f-4dbe-9371-5f2e965598a9
Gudmundsson, Eyjolfur
9c6805d8-0f33-4872-a99f-44f0fbf19bf7
Zhao, An
1c74dd2e-8108-46b7-926f-1810e49bcd6b
Mogulkoc, Nesrin
433ea942-9ac8-4c78-86a3-f1d003043852
Wells, Athol
9e166381-f8f5-4007-968f-380f3663752a
Jones, Mark
a6fd492e-058e-4e84-a486-34c6035429c1
van Moorsel, Coline
bd92cbbd-c977-4eb6-a09a-fa273d8fc368
Porter, Joanna
83ce24e7-4f91-4ce5-9702-fa6a86074c6f
Wuyts, Wim
10be264e-5be4-42f4-9e90-43fa6474f82a
Stewart, Iain
4881bb01-497f-4ff9-8f23-36b513e944ee
Jacob, Joseph
de510fa4-f11f-4dbe-9371-5f2e965598a9

Gudmundsson, Eyjolfur, Zhao, An, Mogulkoc, Nesrin, Wells, Athol, Jones, Mark, van Moorsel, Coline, Porter, Joanna, Wuyts, Wim, Stewart, Iain and Jacob, Joseph (2022) Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis. ERJ Open Research. (In Press)

Record type: Article

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 (∆-PPFE) was calculated. ∆-PPFE >1.25% defined progressive PPFE above scan noise. Mixed-effects models evaluated ∆-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: ∆-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 correlate strongly with measures of fibrosis progression.

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PPFE_Longit_Manuscript_ERJ_2022_Resub_Final - Accepted Manuscript
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Accepted/In Press date: 1 December 2022

Identifiers

Local EPrints ID: 473344
URI: http://eprints.soton.ac.uk/id/eprint/473344
ISSN: 2312-0541
PURE UUID: 9cb38e4b-06a7-46f0-a76c-b0700c875be3
ORCID for Mark Jones: ORCID iD orcid.org/0000-0001-6308-6014

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Date deposited: 16 Jan 2023 17:44
Last modified: 17 Mar 2024 03:12

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Contributors

Author: Eyjolfur Gudmundsson
Author: An Zhao
Author: Nesrin Mogulkoc
Author: Athol Wells
Author: Mark Jones ORCID iD
Author: Coline van Moorsel
Author: Joanna Porter
Author: Wim Wuyts
Author: Iain Stewart
Author: Joseph Jacob

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