{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T23:26:01Z","timestamp":1792193161922,"version":"4.4.0"},"reference-count":26,"publisher":"European Respiratory Society (ERS)","issue":"4","license":[{"start":{"date-parts":[[2023,11,16]],"date-time":"2023-11-16T00:00:00Z","timestamp":1700092800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.ersjournals.com\/user-licence"}],"funder":[{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["209553\/Z\/17\/Z"],"award-info":[{"award-number":["209553\/Z\/17\/Z"]}],"id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["publications.ersnet.org"],"crossmark-restriction":true},"short-container-title":["Eur Respir J"],"accepted":{"date-parts":[[2023,9,24]]},"published-print":{"date-parts":[[2024,4]]},"abstract":"<jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Visual emphysema presence (&gt;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 (\n                      <jats:italic>D<\/jats:italic>\n                      <jats:sub>LCO<\/jats:sub>\n                      ) decline (linear mixed-effects models), and their associations with mortality (multivariable Cox regression models) were compared across non-CPFE and CPFE subgroups.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      In both IPF cohorts, CPFE patients with \u226510% emphysema had a greater smoking history and lower baseline\n                      <jats:italic>D<\/jats:italic>\n                      <jats:sub>LCO<\/jats:sub>\n                      compared to CPFE patients with &lt;10% emphysema. Using multivariable Cox regression analyses in patients with \u226510% emphysema, 1-year\n                      <jats:italic>D<\/jats:italic>\n                      <jats:sub>LCO<\/jats:sub>\n                      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 \u226515% 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 \u22655% and \u226510% showed strong mortality associations.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>\n                      When assessing disease progression in IPF,\n                      <jats:italic>D<\/jats:italic>\n                      <jats:sub>LCO<\/jats:sub>\n                      decline should be considered in patients with \u226510% emphysema and a \u22655% 1-year relative FVC decline threshold considered in non-CPFE IPF patients.\n                    <\/jats:p>\n                  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