ATS 2021 will feature presentations of original research from accepted abstracts. Mini Symposia and Thematic Poster Sessions are abstract based sessions.
Please use the form below to browse scientific abstracts and case reports accepted for ATS 2021. Abstracts presented at the ATS 2021 will be published in the Online Abstract Issue of the American Journal of Respiratory and Critical Care Medicine, Volume 203, May 3, 2021.
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Effects of Visually Scored Pleuroparenchymal Fibroelastosis Severity on Functional Decline and Mortality in IPF Patients
Session Title
TP126 - TP126 STRUCTURE AND FUNCTION IN PARENCHYMAL LUNG DISEASES
Abstract
A4649 - Effects of Visually Scored Pleuroparenchymal Fibroelastosis Severity on Functional Decline and Mortality in IPF Patients
Author Block: E. Gudmundsson1, A. Zhao1, N. Mogulkoc2, M. G. Jones3, C. v. Moorsel4, R. Savas2, C. J. Brereton5, H. W. v. Es4, O. Unat6, K. Pontoppidan3, F. v. Beek4, M. Veltkamp7, B. Gholipour8, S. Janes9, I. Stewart10, D. C. Alexander1, J. Jacob1; 1Centre for Medical Image Computing, UCL, London, United Kingdom, 2Ege University Hospital, Izmir, Turkey, 3University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom, 4St Antonius Hospital, Nieuwegein, Netherlands, 5University of Southampton, Southampton, United Kingdom, 6Ege University, Izmir, Turkey, 7Pulmonology, St. Antonius Hospital, Nieuwegein, Netherlands, 8University College London Hospitals NHS Foundation Trust, London, United Kingdom, 9UCL Respiratory, London, United Kingdom, 10Respiratory Medicine, University of Nottingham, Nottingham, United Kingdom.
Rationale: Pleuroparenchymal fibroelastosis (PPFE) is an upper-lobe predominant disease pattern increasingly associated with interstitial pneumonias such as idiopathic pulmonary fibrosis (IPF). PPFE is predictive of worsening functional status and survival in systemic sclerosis and hypersensitivity pneumonitis patients. The purpose of this study was to investigate the effects of PPFE severity, as scored by a radiologist, on functional decline and mortality in discovery and validation cohorts of IPF patients.
Methods: IPF patients with volumetric CT scans (discovery cohort: n=144 Ege University Hospital, Izmir, Turkey; validation cohort (n=147): n=70 St Antonius Hospital, Utrecht, Netherlands and n=77 University Hospital Southampton NHS Foundation Trust, Southampton, UK) had a disease pattern reflective of PPFE measured on CT imaging. PPFE was identified as pleurally-based triangular areas of high density in accordance with previous studies. Individual lung lobes were rated for PPFE severity on a 4-point Likert scale by a single radiologist: 0 = PPFE absent from lobe, 1 = PPFE affecting <10% of the pleural surface area of the lobe, 2 = affecting 10-33%, 3 = affecting >33% (maximum patient score = 18). Multivariable linear regression models were used to investigate relationships between visual PPFE scores and relative 1-year forced vital capacity (FVC) decline. Multivariable Cox regression analyses were undertaken to investigate relationships between visual PPFE scores and mortality at baseline. Separate models were created for all-lobe visual PPFE scores and upper-lobe visual PPFE severity due to upper predominance of PPFE. PPFE score summed across all lobes was dichotomised at >0 to investigate the effect of PPFE presence; upper-lobe PPFE score was dichotomised at >2 to investigate the effect of marked PPFE in upper lung. All models were adjusted for gender, age at CT, smoking (never/ever), antifibrotic use (never/ever), and baseline percentage predicted diffusing capacity of carbon monoxide (DLco%).
Results: Radiological PPFE was absent from all lobes in 77/144 (53%) patients in the discovery cohort and 41/147 (28%) patients in the validation cohort. In patients with PPFE, the mean summed visual score across all lobes was 3.7 +/- 2.6 (discovery cohort) and 3.2 +/- 2.4 (validation cohort). Upper-lobe PPFE scores independently and positively associated with 1-year FVC decline in multivariable linear regression models and significantly predicted mortality in multivariable Cox regression models in both discovery and validation cohorts (see table).
Conclusion: Radiologist visual scoring of upper-lobe PPFE severity in IPF patients independently predicted 1-year FVC decline and mortality in separate discovery and validation cohorts.
Author Block: E. Gudmundsson1, A. Zhao1, N. Mogulkoc2, M. G. Jones3, C. v. Moorsel4, R. Savas2, C. J. Brereton5, H. W. v. Es4, O. Unat6, K. Pontoppidan3, F. v. Beek4, M. Veltkamp7, B. Gholipour8, S. Janes9, I. Stewart10, D. C. Alexander1, J. Jacob1; 1Centre for Medical Image Computing, UCL, London, United Kingdom, 2Ege University Hospital, Izmir, Turkey, 3University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom, 4St Antonius Hospital, Nieuwegein, Netherlands, 5University of Southampton, Southampton, United Kingdom, 6Ege University, Izmir, Turkey, 7Pulmonology, St. Antonius Hospital, Nieuwegein, Netherlands, 8University College London Hospitals NHS Foundation Trust, London, United Kingdom, 9UCL Respiratory, London, United Kingdom, 10Respiratory Medicine, University of Nottingham, Nottingham, United Kingdom.
Rationale: Pleuroparenchymal fibroelastosis (PPFE) is an upper-lobe predominant disease pattern increasingly associated with interstitial pneumonias such as idiopathic pulmonary fibrosis (IPF). PPFE is predictive of worsening functional status and survival in systemic sclerosis and hypersensitivity pneumonitis patients. The purpose of this study was to investigate the effects of PPFE severity, as scored by a radiologist, on functional decline and mortality in discovery and validation cohorts of IPF patients.
Methods: IPF patients with volumetric CT scans (discovery cohort: n=144 Ege University Hospital, Izmir, Turkey; validation cohort (n=147): n=70 St Antonius Hospital, Utrecht, Netherlands and n=77 University Hospital Southampton NHS Foundation Trust, Southampton, UK) had a disease pattern reflective of PPFE measured on CT imaging. PPFE was identified as pleurally-based triangular areas of high density in accordance with previous studies. Individual lung lobes were rated for PPFE severity on a 4-point Likert scale by a single radiologist: 0 = PPFE absent from lobe, 1 = PPFE affecting <10% of the pleural surface area of the lobe, 2 = affecting 10-33%, 3 = affecting >33% (maximum patient score = 18). Multivariable linear regression models were used to investigate relationships between visual PPFE scores and relative 1-year forced vital capacity (FVC) decline. Multivariable Cox regression analyses were undertaken to investigate relationships between visual PPFE scores and mortality at baseline. Separate models were created for all-lobe visual PPFE scores and upper-lobe visual PPFE severity due to upper predominance of PPFE. PPFE score summed across all lobes was dichotomised at >0 to investigate the effect of PPFE presence; upper-lobe PPFE score was dichotomised at >2 to investigate the effect of marked PPFE in upper lung. All models were adjusted for gender, age at CT, smoking (never/ever), antifibrotic use (never/ever), and baseline percentage predicted diffusing capacity of carbon monoxide (DLco%).
Results: Radiological PPFE was absent from all lobes in 77/144 (53%) patients in the discovery cohort and 41/147 (28%) patients in the validation cohort. In patients with PPFE, the mean summed visual score across all lobes was 3.7 +/- 2.6 (discovery cohort) and 3.2 +/- 2.4 (validation cohort). Upper-lobe PPFE scores independently and positively associated with 1-year FVC decline in multivariable linear regression models and significantly predicted mortality in multivariable Cox regression models in both discovery and validation cohorts (see table).
Conclusion: Radiologist visual scoring of upper-lobe PPFE severity in IPF patients independently predicted 1-year FVC decline and mortality in separate discovery and validation cohorts.
