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S123 Pleuroparenchymal fibroelastosis: clinical, functional and morphologic determinants of mortality

Thorax, 2021-02, Vol.76 (Suppl 1), p.A74-A74 [Peer Reviewed Journal]

Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ. ;2021 Author(s) (or their employer(s)) 2021. No commercial re-use. See rights and permissions. Published by BMJ. ;ISSN: 0040-6376 ;EISSN: 1468-3296 ;DOI: 10.1136/thorax-2020-BTSabstracts.128

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  • Title:
    S123 Pleuroparenchymal fibroelastosis: clinical, functional and morphologic determinants of mortality
  • Author: Chua, F ; Bartlett, EC ; Barnett, J ; Devaraj, A ; Renzoni, E ; Nicholson, AG ; Rice, A ; Molyneaux, P ; George, P ; Kokosi, M ; Kouranos, V ; Maher, TM ; Wells, AU ; Desai, SR
  • Subjects: Mortality
  • Is Part Of: Thorax, 2021-02, Vol.76 (Suppl 1), p.A74-A74
  • Description: Introduction and ObjectivesProgressive pleuroparenchymal fibroelastosis (PPFE) is associated with a high symptom burden and frequently co-exists with a separate interstitial lung disease. The prognostic impact of such combinations is unclear and the clinical and computed tomographic (CT) determinants of mortality remain poorly characterised.MethodsPatients with a diagnosis of PPFE (2004–19) were retrieved from the Royal Brompton Hospital ILD databases. CTs were evaluated for radiologic features, including: 1) the cranio-caudal extent and the severity of PPFE; 2) the hilar position (ratio of the lung apex to the ‘hilar point’ distance/lung apex to the diaphragmatic dome distance); 3) upper lobe volume loss, and 4) presence of co-existent ILD.Results139 patients (75 [54%] female; median age 63.5, IQR 52–71.5) were evaluated, including 51 (36.7%) with idiopathic PPFE, 41 (29.5%) with concomitant idiopathic UIP, 17 (12.2%) with hypersensitivity pneumonitis and 8 (5.8%) with autoimmunity. Histopathological information was available in 50 (36%) patients, including from 39 surgical biopsies. 51 deaths were recorded among 130 patients with longitudinal data, yielding a median survival of 3 years. The mean severity of PPFE was negatively correlated with hilar position (r = -0.38, P<0.0005). Unadjusted hazard ratio (HR) analysis for mortality showed: age (HR 1.03 [95% CI: 1.01–1.05; P <0.01), DLco (HR 0.97 [95% CI: 0.95–0.99; P=0.01), composite physiologic index/CPI (HR 1.04 [95% CI: 1.01–1.07; P<0.005), right and left upper lobe volume loss (HR 0.97 [95% CI: 0.95–0.99]; P <0.01 and HR 0.95 [95% CI: 0.92–0.99; P<0.005, respectively) and the average of the right and left hilar position (HR 1.08 [95% CI: 1.04–1.13; P<0.0005). Although PPFE severity (P<0.001) and co-existent ILD (P<0.0005) were revealed as determinants of the hilar position (R2=0.26), multivariable adjustment confirmed that only CPI (HR, 1.04 [95% CI: 1.00–1.07; P <0.05), increased age (HR, 1.03 [95% CI: 1.01–1.06; P =0.01) and mean hilar position (HR, 1.06 [95% CI, 1.01–1.12; P =0.02) independently predicted mortality.ConclusionsPatients with progressive PPFE have a poor outcome, with a median survival that is comparable to IPF. Identifiable and measurable changes in specific clinical, physiologic and radiologic parameters appear to characterise the adverse prognostic profile of these individuals.
  • Publisher: London: BMJ Publishing Group LTD
  • Language: English
  • Identifier: ISSN: 0040-6376
    EISSN: 1468-3296
    DOI: 10.1136/thorax-2020-BTSabstracts.128
  • Source: Alma/SFX Local Collection
    ProQuest Central
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