Application and performance of disease activity indices proposed for patients with systemic sclerosis in an international cohort of patients with juvenile systemic sclerosis

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dc.contributor.authorKlotsche, Jens
dc.contributor.authorTorok, Kathryn S.
dc.contributor.authorKasapcopur, Ozgur
dc.contributor.authorAdrovic, Amra
dc.contributor.authorTerreri, Maria Teresa
dc.contributor.authorSakamoto, Ana Paula
dc.contributor.authorKatsicas, Maria
dc.contributor.authorSztajnbok, Flavio
dc.contributor.authorMarrani, Edoardo
dc.contributor.authorSifuentes-Giraldo, Alberto
dc.contributor.authorStanevicha, Valda
dc.contributor.authorAnton, Jordi
dc.contributor.authorFeldmann, Brian
dc.contributor.authorKostik, Mikhail
dc.contributor.authorNemcova, Dana
dc.contributor.authorSantos, Maria Jose
dc.contributor.authorAppenzeller, Simone
dc.contributor.authorAvcin, Tadej
dc.contributor.authorBattagliotti, Cristina
dc.contributor.authorBerntson, Lillemor
dc.contributor.authorBica, Blanca
dc.contributor.authorBrunner, Jürgen
dc.contributor.authorEleftheriou, Despina
dc.contributor.authorHarel, Liora
dc.contributor.authorHorneff, Gerd
dc.contributor.authorKallinich, Tilmann
dc.contributor.authorMinden, Kirsten
dc.contributor.authorNielsen, Susan
dc.contributor.authorPatwardhan, Anjali
dc.contributor.authorHelmus, Nicola
dc.contributor.authorFoeldvari, Ivan
dc.contributor.institutionDepartment of Paediatrics
dc.date.accessioned2023-10-05T08:40:01Z
dc.date.available2023-10-05T08:40:01Z
dc.date.issued2023-04-10
dc.descriptionFunding Information: The author(s) disclosed receipt of the following financial support for the research, authorship and/or publication of this article: the inception cohort project is supported by an unrestricted grant from the Joachim Herz Stiftung, Hamburg, Germany. Publisher Copyright: © The Author(s) 2023.
dc.description.abstractObjectives: Juvenile systemic sclerosis is a rare childhood disease. Three disease activity indices have been published for adult patients with systemic sclerosis: the European Scleroderma Study Group Index, a modified version of the European Scleroderma Study Group Index and the revised European Scleroderma Trials and Research index. The objective of this study was to determine the feasibility and performance of the three disease activity indices in a prospectively followed cohort of patients with juvenile systemic sclerosis. Methods: The analysis cohort was selected from the prospective international inception cohort enrolling juvenile systemic sclerosis patients. The correlation of the disease activity indices with the physicians’ and the patients’ global assessment of disease activity was determined. The disease activity indices were compared between patients with active and inactive disease. Sensitivity to change between 6- and 12-month follow-up was investigated by mixed models. Results: Eighty percent of the 70 patients had a diffuse cutaneous subtype. The revised European Scleroderma Trials and Research index was highly correlated with the physician-reported global disease activity/parents-reported global disease activity (r = 0.74/0.64), followed by the European Scleroderma Study Group activity index (r = 0.61/0.55) and the modified version of the European Scleroderma Study Group activity index (r = 0.51/0.43). The disease activity indices significantly differed between active and inactive patients. The disease activity indices showed sensitivity to change between 6- and 12-month follow-up among patients who improved or worsened according to the physician-reported global disease activity and the parents-reported global disease activity. Conclusion: Overall, no disease activity score is superior to the other, and all three scores have limitations in the application in juvenile systemic sclerosis patients. Furthermore, research on the concept of disease activity and suitable scores to measure disease activity in patients with juvenile systemic sclerosis is necessary in future.en
dc.description.statusPeer reviewed
dc.format.extent9
dc.format.extent464142
dc.identifier.citationKlotsche, J, Torok, K S, Kasapcopur, O, Adrovic, A, Terreri, M T, Sakamoto, A P, Katsicas, M, Sztajnbok, F, Marrani, E, Sifuentes-Giraldo, A, Stanevicha, V, Anton, J, Feldmann, B, Kostik, M, Nemcova, D, Santos, M J, Appenzeller, S, Avcin, T, Battagliotti, C, Berntson, L, Bica, B, Brunner, J, Eleftheriou, D, Harel, L, Horneff, G, Kallinich, T, Minden, K, Nielsen, S, Patwardhan, A, Helmus, N & Foeldvari, I 2023, 'Application and performance of disease activity indices proposed for patients with systemic sclerosis in an international cohort of patients with juvenile systemic sclerosis', Journal of Scleroderma and Related Disorders, vol. 8, no. 3, pp. 183-191. https://doi.org/10.1177/23971983231164700
dc.identifier.doi10.1177/23971983231164700
dc.identifier.issn2397-1983
dc.identifier.otherunpaywall: 10.1177/23971983231164700
dc.identifier.urihttps://dspace.rsu.lv/jspui/handle/123456789/14908
dc.identifier.urlhttp://www.scopus.com/inward/record.url?scp=85152465398&partnerID=8YFLogxK
dc.language.isoeng
dc.relation.ispartofJournal of Scleroderma and Related Disorders
dc.rightsinfo:eu-repo/semantics/openAccess
dc.subjectdisease activity
dc.subjectjuvenile systemic sclerosis
dc.subjectSystemic sclerosis
dc.subject3.2 Clinical medicine
dc.subject1.1. Scientific article indexed in Web of Science and/or Scopus database
dc.subjectImmunology and Allergy
dc.subjectRheumatology
dc.subjectImmunology
dc.titleApplication and performance of disease activity indices proposed for patients with systemic sclerosis in an international cohort of patients with juvenile systemic sclerosisen
dc.type/dk/atira/pure/researchoutput/researchoutputtypes/contributiontojournal/article

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