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dc.contributor.authorHetlevik, Siri Opsahl
dc.contributor.authorFlatø, Berit
dc.contributor.authorAaløkken, Trond Mogens
dc.contributor.authorSamersaw-Lund, May Brit
dc.contributor.authorReiseter, Silje
dc.contributor.authorMynarek, Georg
dc.contributor.authorNordal, Ellen Berit
dc.contributor.authorRygg, Marite
dc.contributor.authorLilleby, Vibke
dc.date.accessioned2020-02-26T11:43:55Z
dc.date.available2020-02-26T11:43:55Z
dc.date.issued2019-01-01
dc.description.abstract<i>Objective</i> - To assess the occurrence and extent of interstitial lung disease (ILD) in patients with juvenile mixed connective tissue disease (JMCTD), compare pulmonary function in patients and matched controls, study associations between ILD and disease-related variables, and examine progression of pulmonary manifestations over time.<p> <p><i>Methods</i> - A cohort of 52 patients with JMCTD were examined in a cross-sectional study after a mean 16.2 (SD 10.3) years of disease duration with high-resolution computed tomography (HRCT) and pulmonary function tests (PFT) comprising spirometry, DLCO, and total lung capacity (TLC). Matched controls were examined with PFT. Previous HRCT and PFT were available in 37 and 38 patients (mean 8.8 and 10.3 yrs before study inclusion), respectively.<p> <p><i>Results</i> - Compared to controls, patients with JMCTD had lower forced vital capacity (FVC), DLCO, and TLC (p < 0.01). The most frequent abnormal PFT was DLCO in 67% of patients versus 17% of controls (p < 0.001). Fourteen patients (27%) had ILD on HRCT. Most had ILD in < 10% of their lungs. ILD was associated with low values for FVC and TLC, but not with DLCO. HRCT findings did not progress significantly over time, but FVC declined (p < 0.01).<p> <p><i>Conclusion</i> - Compared to controls, patients with JMCTD had impaired pulmonary function. ILD was present in 27% of patients after a mean 16 years of disease duration, mostly as mild disease, and did not progress. ILD seems to be less common in juvenile-onset than in adult-onset MCTD, and ILD in JMCTD seems mostly mild and stable over time.en_US
dc.descriptionThis is a pre-copyediting, author-produced PDF of an article accepted for publication in The Journal of Rheumatology following peer review. The definitive publisher-authenticated version Hetlevik, S.O., Flatø, B., Aaløkken, T.M., Lund, M.B., Reiseter, S., Mynarek, G.K. ... Lilleby, V. (2019). Pulmonary Manifestations and Progression of Lung Disease in Juvenile-onset Mixed Connective Tissue Disease. <i>The Journal of Rheumatology, 46</i>(1), 93-100, is available online at: https://doi.org/10.3899/jrheum.180019.en_US
dc.identifier.citationHetlevik SO, Flatø B, Aaløkken TM, Samersaw-Lund MB, Reiseter S, Mynarek G, Nordal E, Rygg M, Lilleby V. Pulmonary manifestations and progression of lung disease in juvenile-onset mixed connective tissue disease. Journal of Rheumatology. 2019;46(1):93-100en_US
dc.identifier.cristinIDFRIDAID 1686351
dc.identifier.doi10.3899/jrheum.180019
dc.identifier.issn0315-162X
dc.identifier.issn1499-2752
dc.identifier.urihttps://hdl.handle.net/10037/17514
dc.language.isoengen_US
dc.publisherJournal of Rheumatologyen_US
dc.relation.journalJournal of Rheumatology
dc.rights.accessRightsopenAccessen_US
dc.rights.holderPersonal non-commercial use only. The Journal of Rheumatology Copyright © 2019. All rights reserved.en_US
dc.subjectVDP::Medical disciplines: 700en_US
dc.subjectVDP::Medisinske Fag: 700en_US
dc.titlePulmonary manifestations and progression of lung disease in juvenile-onset mixed connective tissue diseaseen_US
dc.type.versionacceptedVersionen_US
dc.typeJournal articleen_US
dc.typeTidsskriftartikkelen_US
dc.typePeer revieweden_US


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