Pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: Survival analysis using visual and computer-based computed tomography assessment
Background: Idiopathic pulmonary fibrosis (IPF) and pleuroparenchymal fibroelastosis (PPFE) are known to have poor outcomes but detailed examinations of prognostic significance of an association between these morphologic processes are lacking. Methods: Retrospective observational study of independen...
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Elsevier
2021-08-01
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Online Access: | http://www.sciencedirect.com/science/article/pii/S2589537021002893 |
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record_format |
Article |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Eyjolfur Gudmundsson An Zhao Nesrin Mogulkoc Iain Stewart Mark G. Jones Coline H.M. Van Moorsel Recep Savas Christopher J. Brereton Hendrik W. Van Es Omer Unat Katarina Pontoppidan Frouke Van Beek Marcel Veltkamp Bahareh Gholipour Arjun Nair Athol U. Wells Sam M. Janes Daniel C. Alexander Joseph Jacob |
spellingShingle |
Eyjolfur Gudmundsson An Zhao Nesrin Mogulkoc Iain Stewart Mark G. Jones Coline H.M. Van Moorsel Recep Savas Christopher J. Brereton Hendrik W. Van Es Omer Unat Katarina Pontoppidan Frouke Van Beek Marcel Veltkamp Bahareh Gholipour Arjun Nair Athol U. Wells Sam M. Janes Daniel C. Alexander Joseph Jacob Pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: Survival analysis using visual and computer-based computed tomography assessment EClinicalMedicine Pleuroparenchymal fibroelastosis PPFE, Idiopathic pulmonary fibrosis IPF Computed tomography Quantitative analysis |
author_facet |
Eyjolfur Gudmundsson An Zhao Nesrin Mogulkoc Iain Stewart Mark G. Jones Coline H.M. Van Moorsel Recep Savas Christopher J. Brereton Hendrik W. Van Es Omer Unat Katarina Pontoppidan Frouke Van Beek Marcel Veltkamp Bahareh Gholipour Arjun Nair Athol U. Wells Sam M. Janes Daniel C. Alexander Joseph Jacob |
author_sort |
Eyjolfur Gudmundsson |
title |
Pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: Survival analysis using visual and computer-based computed tomography assessment |
title_short |
Pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: Survival analysis using visual and computer-based computed tomography assessment |
title_full |
Pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: Survival analysis using visual and computer-based computed tomography assessment |
title_fullStr |
Pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: Survival analysis using visual and computer-based computed tomography assessment |
title_full_unstemmed |
Pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: Survival analysis using visual and computer-based computed tomography assessment |
title_sort |
pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: survival analysis using visual and computer-based computed tomography assessment |
publisher |
Elsevier |
series |
EClinicalMedicine |
issn |
2589-5370 |
publishDate |
2021-08-01 |
description |
Background: Idiopathic pulmonary fibrosis (IPF) and pleuroparenchymal fibroelastosis (PPFE) are known to have poor outcomes but detailed examinations of prognostic significance of an association between these morphologic processes are lacking. Methods: Retrospective observational study of independent derivation and validation cohorts of IPF populations. Upper-lobe PPFE extent was scored visually (vPPFE) as categories of absent, moderate, marked. Computerised upper-zone PPFE extent (cPPFE) was examined continuously and using a threshold of 2·5% pleural surface area. vPPFE and cPPFE were evaluated against 1-year FVC decline (estimated using mixed-effects models) and mortality. Multivariable models were adjusted for age, gender, smoking history, antifibrotic treatment and diffusion capacity for carbon monoxide. Findings: PPFE prevalence was 49% (derivation cohort, n = 142) and 72% (validation cohort, n = 145). vPPFE marginally contributed 3–14% to variance in interstitial lung disease (ILD) severity across both cohorts.In multivariable models, marked vPPFE was independently associated with 1-year FVC decline (derivation: regression coefficient 18·3, 95 CI 8·47–28·2%; validation: 7·51, 1·85–13·2%) and mortality (derivation: hazard ratio [HR] 7·70, 95% CI 3·50–16·9; validation: HR 3·01, 1·33–6·81). Similarly, continuous and dichotomised cPPFE were associated with 1-year FVC decline and mortality (cPPFE ≥ 2·5% derivation: HR 5·26, 3·00–9·22; validation: HR 2·06, 1·28–3·31). Individuals with cPPFE ≥ 2·5% or marked vPPFE had the lowest median survival, the cPPFE threshold demonstrated greater discrimination of poor outcomes at two and three years than marked vPPFE. Interpretation: PPFE quantification supports distinction of IPF patients with a worse outcome independent of established ILD severity measures. This has the potential to improve prognostic management and elucidate separate pathways of disease progression. Funding: This research was funded in whole or in part by the Wellcome Trust [209,553/Z/17/Z] and the NIHR UCLH Biomedical Research Centre, UK. |
topic |
Pleuroparenchymal fibroelastosis PPFE, Idiopathic pulmonary fibrosis IPF Computed tomography Quantitative analysis |
url |
http://www.sciencedirect.com/science/article/pii/S2589537021002893 |
work_keys_str_mv |
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doaj-0adf401fc86f4683bd4a4ef3183d23932021-08-28T04:48:04ZengElsevierEClinicalMedicine2589-53702021-08-0138101009Pleuroparenchymal fibroelastosis in idiopathic pulmonary fibrosis: Survival analysis using visual and computer-based computed tomography assessmentEyjolfur Gudmundsson0An Zhao1Nesrin Mogulkoc2Iain Stewart3Mark G. Jones4Coline H.M. Van Moorsel5Recep Savas6Christopher J. Brereton7Hendrik W. Van Es8Omer Unat9Katarina Pontoppidan10Frouke Van Beek11Marcel Veltkamp12Bahareh Gholipour13Arjun Nair14Athol U. Wells15Sam M. Janes16Daniel C. Alexander17Joseph Jacob18Centre for Medical Image Computing, UCL, 1st Floor, 90 High Holborn, London WC1V6LJ, United KingdomCentre for Medical Image Computing, UCL, 1st Floor, 90 High Holborn, London WC1V6LJ, United KingdomDepartment of Respiratory Medicine, Ege University Hospital, Izmir, TurkeyNational Heart & Lung Institute, Imperial College London, London, United KingdomNIHR Southampton Biomedical Research Centre and Clinical and Experimental Sciences, University of Southampton, Southampton, United KingdomDepartment of Pulmonology, Interstitial Lung Diseases Center of Excellence, St Antonius Hospital, Nieuwegein, the NetherlandsDepartment of Radiology, Ege University Hospital, Izmir, TurkeyNIHR Southampton Biomedical Research Centre and Clinical and Experimental Sciences, University of Southampton, Southampton, United KingdomDepartment of Radiology, St Antonius Hospital, Nieuwegein, the NetherlandsDepartment of Respiratory Medicine, Ege University Hospital, Izmir, TurkeyNIHR Southampton Biomedical Research Centre and Clinical and Experimental Sciences, University of Southampton, Southampton, United KingdomDepartment of Pulmonology, Interstitial Lung Diseases Center of Excellence, St Antonius Hospital, Nieuwegein, the NetherlandsDepartment of Pulmonology, Interstitial Lung Diseases Center of Excellence, St Antonius Hospital, Nieuwegein, the Netherlands; Division of Heart and Lungs, University Medical Center, Utrecht, the NetherlandsDepartment of Radiology, University College London Hospitals NHS Foundation Trust, London, United KingdomDepartment of Radiology, University College London Hospitals NHS Foundation Trust, London, United KingdomInterstitial Lung Disease Unit, Royal Brompton Hospital, Imperial College, London, United KingdomLungs for Living Research Centre, UCL, London, United KingdomCentre for Medical Image Computing, UCL, 1st Floor, 90 High Holborn, London WC1V6LJ, United KingdomCentre for Medical Image Computing, UCL, 1st Floor, 90 High Holborn, London WC1V6LJ, United Kingdom; Lungs for Living Research Centre, UCL, London, United Kingdom; Corresponding author at: Centre for Medical Image Computing, UCL, 1st Floor, 90 High Holborn, London WC1V6LJ, United Kingdom.Background: Idiopathic pulmonary fibrosis (IPF) and pleuroparenchymal fibroelastosis (PPFE) are known to have poor outcomes but detailed examinations of prognostic significance of an association between these morphologic processes are lacking. Methods: Retrospective observational study of independent derivation and validation cohorts of IPF populations. Upper-lobe PPFE extent was scored visually (vPPFE) as categories of absent, moderate, marked. Computerised upper-zone PPFE extent (cPPFE) was examined continuously and using a threshold of 2·5% pleural surface area. vPPFE and cPPFE were evaluated against 1-year FVC decline (estimated using mixed-effects models) and mortality. Multivariable models were adjusted for age, gender, smoking history, antifibrotic treatment and diffusion capacity for carbon monoxide. Findings: PPFE prevalence was 49% (derivation cohort, n = 142) and 72% (validation cohort, n = 145). vPPFE marginally contributed 3–14% to variance in interstitial lung disease (ILD) severity across both cohorts.In multivariable models, marked vPPFE was independently associated with 1-year FVC decline (derivation: regression coefficient 18·3, 95 CI 8·47–28·2%; validation: 7·51, 1·85–13·2%) and mortality (derivation: hazard ratio [HR] 7·70, 95% CI 3·50–16·9; validation: HR 3·01, 1·33–6·81). Similarly, continuous and dichotomised cPPFE were associated with 1-year FVC decline and mortality (cPPFE ≥ 2·5% derivation: HR 5·26, 3·00–9·22; validation: HR 2·06, 1·28–3·31). Individuals with cPPFE ≥ 2·5% or marked vPPFE had the lowest median survival, the cPPFE threshold demonstrated greater discrimination of poor outcomes at two and three years than marked vPPFE. Interpretation: PPFE quantification supports distinction of IPF patients with a worse outcome independent of established ILD severity measures. This has the potential to improve prognostic management and elucidate separate pathways of disease progression. Funding: This research was funded in whole or in part by the Wellcome Trust [209,553/Z/17/Z] and the NIHR UCLH Biomedical Research Centre, UK.http://www.sciencedirect.com/science/article/pii/S2589537021002893Pleuroparenchymal fibroelastosisPPFE, Idiopathic pulmonary fibrosisIPFComputed tomographyQuantitative analysis |