{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,7]],"date-time":"2026-11-07T16:41:56Z","timestamp":1794069716420,"version":"4.8.0"},"reference-count":32,"publisher":"Oxford University Press (OUP)","issue":"12","license":[{"start":{"date-parts":[[2018,12,1]],"date-time":"2018-12-01T00:00:00Z","timestamp":1543622400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2018,12,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Rationale<\/jats:title>\n                    <jats:p>Previous studies have suggested that interstitial lung disease (ILD) progresses most rapidly early in the course of systemic sclerosis\u2013associated (SSc)-ILD, and that SSc-ILD is often more stable or even \u201cburned out\u201d after the first 4 years following diagnosis.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Our objectives were to determine whether an apparent plateau in pulmonary function decline is due to survival bias and to identify distinct prognostic phenotypes of ILD progression.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Consecutive patients with SSc-ILD from a single center were included. Pulmonary function measurements were typically performed every 6 months. Study participants were categorized into long-term survivors (&amp;gt;8 yr survival from diagnosis), and those with medium-term and short-term mortality (4\u20138 and &amp;lt;4 yr survival, respectively). We excluded those censored with less than 8 years of follow-up. Subject-specific slopes for change in forced vital capacity (FVC) and diffusing capacity of the lung for carbon monoxide (DlCO) were calculated using generalized linear models with mixed effects. The rate of decline in FVC was compared across prognostic groups.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The cohort included 171 study participants with SSc-ILD. A plateau in the progression of FVC was apparent in the full cohort analysis but disappeared with stratification into prognostic subgroups to account for survival bias. Those with short-term mortality had a higher annual rate of decline in FVC (\u22124.10 [95% confidence interval (CI), \u22127.92 to \u22120.28] vs. \u22122.14 [95% CI, \u22123.31 to \u22120.97] and \u22120.94 [\u22121.46 to \u22120.42]; P\u2009=\u20090.003) and DlCO (\u22125.28 [95% CI, \u22129.58 to \u22120.99] vs. \u22123.13 [95% CI, \u22124.35 to \u22121.92] and \u22121.32 [95% CI, \u22122.01 to \u22120.63]; P\u2009&amp;lt;\u20090.001) than those with medium-term mortality and long-term survival with adjustment for age, sex, and pack-years. Change in FVC in the previous year did not predict FVC change in the subsequent year.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Adults with SSc-ILD have distinct patterns of physiological progression that remain relatively consistent during long-term follow-up; however, recent change in FVC cannot be used to predict future change in FVC within shorter follow-up intervals. The findings of this study provide important information on the course of disease in SSc-ILD and identify specific phenotypes of progression that may improve clinical decision-making and design of future therapeutic trials.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1513\/annalsats.201806-362oc","type":"journal-article","created":{"date-parts":[[2018,9,6]],"date-time":"2018-09-06T12:57:01Z","timestamp":1536238621000},"page":"1427-1433","source":"Crossref","is-referenced-by-count":86,"title":["Does Systemic Sclerosis\u2013Associated Interstitial Lung Disease Burn Out? Specific Phenotypes of Disease Progression"],"prefix":"10.1093","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9833-5911","authenticated-orcid":false,"given":"Sabina A.","family":"Guler","sequence":"first","affiliation":[{"name":"Department of Medicine"},{"name":"Centre for Heart Lung Innovation"},{"name":"Department of Pulmonary Medicine, University Hospital and University of Bern, Bern, Switzerland; and"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tiffany A.","family":"Winstone","sequence":"additional","affiliation":[{"name":"Department of Medicine"},{"name":"Centre for Heart Lung Innovation"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Darra","family":"Murphy","sequence":"additional","affiliation":[{"name":"Department of Radiology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cameron","family":"Hague","sequence":"additional","affiliation":[{"name":"Department of Radiology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeanette","family":"Soon","sequence":"additional","affiliation":[{"name":"Department of Radiology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nada","family":"Sulaiman","sequence":"additional","affiliation":[{"name":"Department of Radiology"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathy H.","family":"Li","sequence":"additional","affiliation":[{"name":"Centre for Health Evaluation and Outcome Sciences, Providence Healthcare, St. Paul\u2019s Hospital, Vancouver, British Columbia, Canada"},{"name":"Collaboration for Outcomes Research and Evaluation, Faculty of Pharmaceutical Sciences, University of British Columbia, Vancouver, British Columbia, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James","family":"Dunne","sequence":"additional","affiliation":[{"name":"Department of Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pearce G.","family":"Wilcox","sequence":"additional","affiliation":[{"name":"Department of Medicine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christopher J.","family":"Ryerson","sequence":"additional","affiliation":[{"name":"Department of Medicine"},{"name":"Centre for Heart Lung Innovation"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2018,12,1]]},"reference":[{"key":"2026020502210696000_bib1","doi-asserted-by":"crossref","first-page":"754","DOI":"10.1136\/ard.2006.062901","article-title":"Clinical risk assessment of organ manifestations in systemic sclerosis: a report from the EULAR Scleroderma Trials and Research Group database","volume":"66","author":"Walker","year":"2007","journal-title":"Ann Rheum 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