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Mortality surrogates in combined pulmonary fibrosis and emphysema

Title: Mortality surrogates in combined pulmonary fibrosis and emphysema
Authors: Zhao, An; Gudmundsson, Eyjolfur; Mogulkoc, Nesrin; van Moorsel, Coline; Corte, Tamera J.; Vasudev, Pardeep; Romei, Chiara; Chapman, Robert; Wallis, Tim J.M.; Denneny, Emma; Goos, Tinne; Savas, Recep; Ahmed, Asia; Brereton, Christopher J.; van Es, Hendrik W.; Jo, Helen; De Liperi, Annalisa; Duncan, Mark; Pontoppidan, Katarina; De Sadeleer, Laurens J.; van Beek, Frouke; Barnett, Joseph; Cross, Gary; Procter, Alex; Veltkamp, Marcel; Hopkins, Peter; Moodley, Yuben; Taliani, Alessandro; Taylor, Magali; Verleden, Stijn; Tavanti, Laura; Vermant, Marie; Nair, Arjun; Stewart, Iain; Janes, Sam M.; Young, Alexandra L.; Barber, David; Alexander, Daniel C.; Porter, Joanna C.; Wells, Athol U.; Jones, Mark G.; Wuyts, Wim A.; Jacob, Joseph
Source: 0903-1936 ; The European respiratory journal
Publication Year: 2024
Collection: IRUA - Institutional Repository van de Universiteit Antwerpen
Subject Terms: Human medicine
Description: Background: Idiopathic pulmonary fibrosis (IPF) with coexistent emphysema, termed combined pulmonary fibrosis and emphysema (CPFE) may associate with reduced forced vital capacity (FVC) declines compared to non-CPFE IPF patients. We examined associations between mortality and functional measures of disease progression in two IPF cohorts. Methods: Visual emphysema presence (>0% emphysema) scored on computed tomography identified CPFE patients (CPFE/non-CPFE: derivation cohort n=317/n=183, replication cohort n=358/n=152), who were subgrouped using 10% or 15% visual emphysema thresholds, and an unsupervised machine-learning model considering emphysema and interstitial lung disease extents. Baseline characteristics, 1-year relative FVC and diffusing capacity of the lung for carbon monoxide (D (LCO)) decline (linear mixed-effects models), and their associations with mortality (multivariable Cox regression models) were compared across non-CPFE and CPFE subgroups. Results: In both IPF cohorts, CPFE patients with >= 10% emphysema had a greater smoking history and lower baseline D (LCO) compared to CPFE patients with = 10% emphysema, 1-year D (LCO) decline showed stronger mortality associations than 1-year FVC decline. Results were maintained in patients suitable for therapeutic IPF trials and in subjects subgrouped by >= 15% emphysema and using unsupervised machine learning. Importantly, the unsupervised machine-learning approach identified CPFE patients in whom FVC decline did not associate strongly with mortality. In non-CPFE IPF patients, 1-year FVC declines >= 5% and >= 10% showed strong mortality associations. Conclusion: When assessing disease progression in IPF, D (LCO) decline should be considered in patients with >= 10% emphysema and a >= 5% 1-year relative FVC decline threshold considered in non-CPFE IPF patients.
Document Type: article in journal/newspaper
Language: English
Relation: info:eu-repo/semantics/altIdentifier/isi/001208899300007
Availability: https://hdl.handle.net/10067/2060530151162165141; https://repository.uantwerpen.be/docstore/d:irua:23698
Rights: info:eu-repo/semantics/openAccess
Accession Number: edsbas.D4F2BA30
Database: BASE