{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,4]],"date-time":"2026-09-04T21:12:39Z","timestamp":1788556359230,"version":"build-2803163510"},"reference-count":33,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2022,2,27]],"date-time":"2022-02-27T00:00:00Z","timestamp":1645920000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Respirology"],"published-print":{"date-parts":[[2022,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and objective<\/jats:title><jats:p>Demographic and clinical variables, measured at baseline or over time, have been associated with mortality in subjects with progressive fibrosing interstitial lung diseases (ILDs). We used data from the INPULSIS trials in subjects with idiopathic pulmonary fibrosis (IPF) and the INBUILD trial in subjects with other progressive fibrosing ILDs to assess relationships between demographic\/clinical variables and mortality.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>The relationships between baseline variables and time\u2010varying covariates and time to death over 52\u2009weeks were analysed using pooled data from the INPULSIS trials and, separately, the INBUILD trial using a Cox proportional hazards model.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Over 52\u2009weeks, 68\/1061 (6.4%) and 33\/663 (5.0%) subjects died in the INPULSIS and INBUILD trials, respectively. In the INPULSIS trials, a relative decline in forced vital capacity (FVC) &gt;10% predicted within 12\u2009months (hazard ratio [HR] 3.77) and age (HR 1.03 per 1\u2010year increase) were associated with increased risk of mortality, while baseline FVC % predicted (HR 0.97 per 1\u2010unit increase) and diffusing capacity of the lungs for carbon monoxide (DLCO) % predicted (HR 0.77 per 1\u2010unit increase) were associated with lower risk. In the INBUILD trial, a relative decline in FVC &gt;10% predicted within 12\u2009months (HR 2.60) and a usual interstitial pneumonia\u2010like fibrotic pattern on HRCT (HR 2.98) were associated with increased risk of mortality, while baseline DLCO % predicted (HR 0.95 per 1\u2010unit increase) was associated with lower risk.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>These data support similarity in the course of lung injury between IPF and other progressive fibrosing ILDs and the value of FVC decline as a predictor of mortality.<\/jats:p><\/jats:sec>","DOI":"10.1111\/resp.14231","type":"journal-article","created":{"date-parts":[[2022,2,28]],"date-time":"2022-02-28T04:27:47Z","timestamp":1646022467000},"page":"294-300","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":47,"title":["Predictors of mortality in subjects with progressive fibrosing interstitial lung diseases"],"prefix":"10.1111","volume":"27","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9209-3261","authenticated-orcid":false,"given":"Kevin K.","family":"Brown","sequence":"first","affiliation":[{"name":"Department of Medicine National Jewish Health  Denver Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3994-874X","authenticated-orcid":false,"given":"Yoshikazu","family":"Inoue","sequence":"additional","affiliation":[{"name":"Clinical Research Center National Hospital Organization Kinki\u2010Chuo Chest Medical Center  Sakai City Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin R.","family":"Flaherty","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Critical Care Medicine University of Michigan  Ann Arbor Michigan USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fernando J.","family":"Martinez","sequence":"additional","affiliation":[{"name":"Weill Cornell Medicine  New York New York USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5591-0955","authenticated-orcid":false,"given":"Vincent","family":"Cottin","sequence":"additional","affiliation":[{"name":"National Reference Centre for Rare Pulmonary Diseases, Louis Pradel Hospital, Hospices Civils de Lyon Claude Bernard University Lyon 1  Lyon France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7579-9767","authenticated-orcid":false,"given":"Francesco","family":"Bonella","sequence":"additional","affiliation":[{"name":"Center for Interstitial and Rare Lung Diseases, Department of Pneumology, Ruhrlandklinik University Hospital University of Duisburg\u2010Essen  Essen Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefania","family":"Cerri","sequence":"additional","affiliation":[{"name":"Center for Rare Lung Disease Azienda Ospedaliero\u2010Universitaria Policlinico di Modena  Modena Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sonye K.","family":"Danoff","sequence":"additional","affiliation":[{"name":"Johns Hopkins Medicine  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1949-3461","authenticated-orcid":false,"given":"Stephane","family":"Jouneau","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine Competences Centre for Rare Pulmonary Diseases, CHU Rennes, IRSET UMR 1085, Univ Rennes  Rennes France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rainer\u2010Georg","family":"Goeldner","sequence":"additional","affiliation":[{"name":"Boehringer Ingelheim Pharma GmbH &amp; Co. 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