{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T02:11:37Z","timestamp":1788833497081,"version":"build-2803163510"},"reference-count":34,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T00:00:00Z","timestamp":1788739200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,9,7]],"date-time":"2026-09-07T00:00:00Z","timestamp":1788739200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100031627","name":"Boehringer Ingelheim Canada","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100031627","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Respirology"],"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background and Objective<\/jats:title>\n                    <jats:p>This study determined contemporary transplant\u2010free survival in fibrotic interstitial lung disease (ILD), identified baseline factors associated with death or transplant, and assessed performance of the ILD\u2010GAP (Gender, Age, Physiology) index in a contemporary cohort.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Patients with idiopathic pulmonary fibrosis (IPF), fibrotic hypersensitivity pneumonitis (fHP), connective tissue disease\u2013associated ILD (CTD\u2010ILD), or unclassifiable ILD (uILD) prospectively enrolled in the Canadian Registry for Pulmonary Fibrosis were included. Transplant\u2010free survival at 1, 3, and 5\u2009years was estimated overall and by subtype using enrollment as time zero. Cox proportional hazards models evaluated associations between patient characteristics and time to death or transplant. ILD\u2010GAP scores were calculated at 1, 2, and 3\u2009years, and discriminability was assessed with Harrell's C statistic.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Among 4236 patients with fibrotic ILD enrolled in the Canadian Registry for Pulmonary Fibrosis (CARE\u2010PF), 31% had IPF, 40% CTD\u2010ILD, 9% fHP, and 20% uILD. Overall transplant\u2010free survival was 91% at 1\u2009year, 72% at 3\u2009years, and 57% at 5\u2009years. Survival was lowest in IPF (87%, 61%, 39%) and highest in CTD\u2010ILD (95%, 81%, 70%). Compared with IPF, CTD\u2010ILD was associated with better 5\u2010year survival after adjustment (hazard ratio 0.64 [0.55\u20130.76]). ILD\u2010GAP showed moderate discrimination at 1 (C\u2009=\u20090.75), 2 (C\u2009=\u20090.74), and 3\u2009years (C\u2009=\u20090.73).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In this registry cohort anchored at enrollment, CTD\u2010ILD had superior transplant\u2010free survival versus IPF, and ILD\u2010GAP provided moderate prognostic accuracy.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/resp.70305","type":"journal-article","created":{"date-parts":[[2026,9,8]],"date-time":"2026-09-08T02:00:25Z","timestamp":1788832825000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Contemporary Fibrotic Interstitial Lung Disease Outcomes: A Multicentre National Cohort Study"],"prefix":"10.1002","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3015-0723","authenticated-orcid":false,"given":"Tyler","family":"Pitre","sequence":"first","affiliation":[{"name":"Department of Medicine University of Toronto  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1049-393X","authenticated-orcid":false,"given":"Christopher J.","family":"Ryerson","sequence":"additional","affiliation":[{"name":"Department of Medicine University of British Columbia  Vancouver British Columbia Canada"},{"name":"Centre for Heart Lung Innovation St. Paul's Hospital  Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shaun","family":"Ong","sequence":"additional","affiliation":[{"name":"Division of Respiratory Medicine, Department of Medicine University of British Columbia  Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kerri A.","family":"Johannson","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Calgary  Calgary Alberta Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deborah","family":"Assayag","sequence":"additional","affiliation":[{"name":"Department of Medicine McGill University  Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5890-3227","authenticated-orcid":false,"given":"Veronica","family":"Marcoux","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Saskatchewan  Saskatoon Saskatchewan Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3837-1467","authenticated-orcid":false,"given":"Martin","family":"Kolb","sequence":"additional","affiliation":[{"name":"Department of Medicine Firestone Institute for Respiratory Health, McMaster University  Hamilton Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Helene","family":"Manganas","sequence":"additional","affiliation":[{"name":"D\u00e9partement de M\u00e9decine Centre Hospitalier de l'Universit\u00e9 de Montr\u00e9al  Montreal Quebec Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3358-7699","authenticated-orcid":false,"given":"Daniel\u2010Costin","family":"Marinescu","sequence":"additional","affiliation":[{"name":"Department of Medicine University of British Columbia  Vancouver British Columbia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8123-923X","authenticated-orcid":false,"given":"Jolene H.","family":"Fisher","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Toronto  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,9,7]]},"reference":[{"issue":"10","key":"e_1_2_11_2_1","doi-asserted-by":"crossref","first-page":"958","DOI":"10.1056\/NEJMra2005230","article-title":"Spectrum of Fibrotic Lung Diseases","volume":"383","author":"Wijsenbeek M.","year":"2020","journal-title":"New England Journal of Medicine"},{"issue":"1","key":"e_1_2_11_3_1","doi-asserted-by":"crossref","DOI":"10.1183\/13993003.02420-2017","article-title":"The Increasing Mortality of Idiopathic Pulmonary Fibrosis: Fact or Fallacy?","volume":"51","author":"Ryerson C. 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G. J. P.","year":"2022","journal-title":"Respiratory Medicine"},{"issue":"2","key":"e_1_2_11_32_1","doi-asserted-by":"crossref","first-page":"L233","DOI":"10.1152\/ajplung.00083.2023","article-title":"Tobacco Smoking Is Associated With Combined Pulmonary Fibrosis and Emphysema and Worse Outcomes in Interstitial Lung Disease","volume":"325","author":"Douglas D.","year":"2023","journal-title":"American Journal of Physiology. Lung Cellular and Molecular Physiology"},{"issue":"1","key":"e_1_2_11_33_1","doi-asserted-by":"crossref","first-page":"4784","DOI":"10.1038\/s41598-018-23073-3","article-title":"Comparison of CPI and GAP Models in Patients With Idiopathic Pulmonary Fibrosis: A Nationwide Cohort Study","volume":"8","author":"Lee S. 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