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dc.contributor.authorHoffmann-Vold, Anna-Maria
dc.contributor.authorFretheim, Håvard Halland
dc.contributor.authorHalse, Anne-Kristine
dc.contributor.authorSeip, Marit
dc.contributor.authorBitter, Helle
dc.contributor.authorWallenius, Marianne
dc.contributor.authorGaren, Torhild Oddveig
dc.contributor.authorSalberg, Anne
dc.contributor.authorBrunborg, Cathrine
dc.contributor.authorMidtvedt, Øyvind
dc.contributor.authorLund, May-Brith
dc.contributor.authorAaløkken, Trond M.
dc.contributor.authorMolberg, Øyvind
dc.date.accessioned2020-09-25T11:00:35Z
dc.date.available2020-09-25T11:00:35Z
dc.date.created2019-11-19T11:59:30Z
dc.date.issued2019
dc.identifier.citationAmerican Journal of Respiratory and Critical Care Medicine. 2019, 200 (10), 1258-1266.en_US
dc.identifier.issn1073-449X
dc.identifier.urihttps://hdl.handle.net/11250/2679685
dc.description.abstractRationale: Interstitial lung disease (ILD) represents a major challenge in systemic sclerosis (SSc), but there are no precise, population-based data on its overall impact, limiting opportunities for screening and management strategies. Objectives: Evaluate impact of ILD in a unique, nationwide, population-based SSc cohort. Methods: ILD was assessed prospectively in the Norwegian SSc (Nor-SSc) cohort, including all 815 patients with SSc resident in the country from 2000 to 2012. Lung high-resolution computed tomography (HRCT) scans were available for fibrosis quantification at baseline (n = 650, 80%) and follow-up. Pulmonary function tests were assessed at baseline (n = 703, 86%) and follow-up. Vital status and standardized mortality ratios (SMRs) were estimated at study end (2018) in the 630 incident Nor-SSc cases and 15 individually matched control subjects. Cumulative survival rates were computed. Measurements and Main Results: At baseline, 50% of the subjects with SSc (n = 324) had ILD by HRCT and 46% displayed pulmonary function declines consistent with ILD progression. Mortality correlated with extent of lung fibrosis as SMR increased from 2.2 with no fibrosis to 8.0 with greater than 25% fibrosis. SMR was inversely related to baseline FVC% and increased at all FVC levels below 100%. In patients with normal-range baseline FVC (80–100%), the 5- and 10-year survival rates correlated with presence or absence of lung fibrosis, being 83% and 80%, respectively, with no fibrosis and 69% and 56%, respectively, with lung fibrosis (P = 0.03). Conclusions: The mere presence of ILD at baseline appears to affect outcome in SSc, suggesting that all patients with SSc should undergo a baseline pulmonary function test and lung HRCT screening to diagnose ILD early and tailor further management.en_US
dc.description.sponsorshipSupported by the Norwegian Women’s Public Health Association and the South Eastern Regional Health Authorities.en_US
dc.language.isoengen_US
dc.subjectautoimmune disease;en_US
dc.subjectepidemiology;en_US
dc.subjectpulmonary fibrosis;en_US
dc.subjectsystemic sclerosis.en_US
dc.titleTracking impact of interstitial lung disease in systemic sclerosis in a complete nationwide cohorten_US
dc.typePeer revieweden_US
dc.typeJournal articleen_US
dc.description.versionacceptedVersionen_US
dc.source.pagenumber1258-1266en_US
dc.source.volume200en_US
dc.source.journalAmerican Journal of Respiratory and Critical Care Medicineen_US
dc.source.issue10en_US
dc.identifier.doi10.1164/rccm.201903-0486OC
dc.identifier.cristin1749330
cristin.ispublishedtrue
cristin.fulltextpostprint
cristin.qualitycode2


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