Zhao, An;
Gudmundsson, Eyjolfur;
Mogulkoc, Nesrin;
van Moorsel, Coline;
Corte, Tamera J;
Vasudev, Pardeep;
Romei, Chiara;
... Jacob, Joseph; + view all
(2023)
Mortality surrogates in combined pulmonary fibrosis and emphysema.
European Respiratory Journal
, 63
(4)
, Article 2300127. 10.1183/13993003.00127-2023.
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Abstract
BACKGROUND: Idiopathic pulmonary fibrosis (IPF) with co-existent 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=317:183; replication cohort=358:152), who were subgrouped using 10%, or 15% visual emphysema thresholds, and an unsupervised machine learning model considering emphysema and ILD extents. Baseline characteristics, 1-year relative FVC and diffusing capacity of the lung for carbon monoxide (DLco) 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 DLco compared to CPFE patients with <10% emphysema. Using multivariable Cox regression analyses in patients with ≥10% emphysema, 1-year DLco 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, DLco decline should be considered in patients with ≥10% emphysema and a ≥5% 1-year relative FVC decline threshold considered in non-CPFE IPF patients.
| Type: | Article |
|---|---|
| Title: | Mortality surrogates in combined pulmonary fibrosis and emphysema |
| Location: | England |
| Open access status: | An open access version is available from UCL Discovery |
| DOI: | 10.1183/13993003.00127-2023 |
| Publisher version: | http://doi.org/10.1183/13993003.00127-2023 |
| Language: | English |
| Additional information: | This version is the author accepted manuscript. - This research was funded in whole or in part by the Wellcome Trust [209553/Z/17/Z]. For the purpose of open access, the author has applied a CC-BY public copyright licence to any author accepted manuscript version arising from this submission. |
| Keywords: | Combined pulmonary fibrosis and emphysema, mortality surrogates, idiopathic pulmonary fibrosis, computed tomography |
| UCL classification: | UCL UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences UCL > Provost and Vice Provost Offices > UCL BEAMS UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences UCL > Provost and Vice Provost Offices > UCL BEAMS > Faculty of Engineering Science UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine UCL > Provost and Vice Provost Offices > UCL BEAMS > Faculty of Engineering Science > Dept of Computer Science UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Respiratory Medicine |
| URI: | https://discovery-pp.ucl.ac.uk/id/eprint/10181920 |
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