{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T21:26:18Z","timestamp":1792531578929,"version":"4.5.0"},"reference-count":23,"publisher":"BMJ","issue":"3","license":[{"start":{"date-parts":[[2023,4,10]],"date-time":"2023-04-10T00:00:00Z","timestamp":1681084800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Scleroderma and Related Disorders"],"published-print":{"date-parts":[[2023,10]]},"abstract":"<jats:sec>\n                    <jats:title>Objectives:<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>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\u2019 and the patients\u2019 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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>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\u2009=\u20090.74\/0.64), followed by the European Scleroderma Study Group activity index (r\u2009=\u20090.61\/0.55) and the modified version of the European Scleroderma Study Group activity index (r\u2009=\u20090.51\/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.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>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.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/23971983231164700","type":"journal-article","created":{"date-parts":[[2023,4,10]],"date-time":"2023-04-10T05:23:02Z","timestamp":1681104182000},"page":"183-191","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":8,"title":["Application and performance of disease activity indices proposed for patients with systemic sclerosis in an international cohort of patients with juvenile systemic sclerosis"],"prefix":"10.1136","volume":"8","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2954-5755","authenticated-orcid":false,"given":"Jens","family":"Klotsche","sequence":"first","affiliation":[{"name":"German Rheumatism Research Center, A Leibniz Institute, Berlin, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathryn S","family":"Torok","sequence":"additional","affiliation":[{"name":"University of Pittsburgh, Children\u2019s Hospital of Pittsburgh, Pittsburgh, PA, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ozgur","family":"Kasapcopur","sequence":"additional","affiliation":[{"name":"Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amra","family":"Adrovic","sequence":"additional","affiliation":[{"name":"Department of Pediatric Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Teresa","family":"Terreri","sequence":"additional","affiliation":[{"name":"Universidade Federal de S\u00e3o Paulo, Sao Paulo, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ana Paula","family":"Sakamoto","sequence":"additional","affiliation":[{"name":"Universidade Federal de S\u00e3o Paulo, Sao Paulo, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Katsicas","sequence":"additional","affiliation":[{"name":"Hospital de Pediatria J.P. Garrahan, Buenos Aires, Argentine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Flavio","family":"Sztajnbok","sequence":"additional","affiliation":[{"name":"Universidade do Estado, Rio de Janeiro, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Edoardo","family":"Marrani","sequence":"additional","affiliation":[{"name":"Meyer Children\u2019s Hospital, Florence, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alberto","family":"Sifuentes-Giraldo","sequence":"additional","affiliation":[{"name":"University Hospital Ram\u00f3n y Cajal, Madrid, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Valda","family":"Stanevicha","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Riga Stradins University, University Children Hospital, Riga, Latvia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8792-4219","authenticated-orcid":false,"given":"Jordi","family":"Anton","sequence":"additional","affiliation":[{"name":"Pediatric Rheumatology, Hospital Sant Joan de D\u00e9u, Universitat de Barcelona, Barcelona, Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brian","family":"Feldmann","sequence":"additional","affiliation":[{"name":"SickKids, The Hospital for Sick Children, Toronto, ON, Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mikhail","family":"Kostik","sequence":"additional","affiliation":[{"name":"Saint-Petersburg State Pediatric Medical University, Saint Petersburg, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dana","family":"Nemcova","sequence":"additional","affiliation":[{"name":"Charles University, Prague, Czech Republic"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Jose","family":"Santos","sequence":"additional","affiliation":[{"name":"Servi\u00e7o de Reumatologia, Hospital Garcia de Orta, Almada, Portugal"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simone","family":"Appenzeller","sequence":"additional","affiliation":[{"name":"School of Medical Science, State University of Campinas, Campinas, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tadej","family":"Avcin","sequence":"additional","affiliation":[{"name":"University Children\u2019s Hospital, University Medical Center Ljubljana, Ljubljana, Slovenia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Battagliotti","sequence":"additional","affiliation":[{"name":"Hospital de Ni\u00f1os Dr Orlando Alassia, Santa Fe, Argentine"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lillemor","family":"Berntson","sequence":"additional","affiliation":[{"name":"Department of Women\u2019s and Children\u2019s Health, Uppsala University, Uppsala, Sweden"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Blanca","family":"Bica","sequence":"additional","affiliation":[{"name":"Hospital Universit\u00e1rio Clementino Fraga Filho (HUCFF), Universidade Federal do Rio de Janeiro, Rio de Janeiro, Brazil"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00fcrgen","family":"Brunner","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Pediatric Rheumatology, Medical University Innsbruck, Innsbruck, Austria"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Despina","family":"Eleftheriou","sequence":"additional","affiliation":[{"name":"Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Liora","family":"Harel","sequence":"additional","affiliation":[{"name":"Schneider Children\u2019s Medical Center, Tel Aviv University, Petah Tikva, 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