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Recently, we identified 2 proteins, galectin\u20109 and CXCL<\/jats:styled-content>10, whose levels are highly correlated with the extent of juvenile DM<\/jats:styled-content> disease activity. This study was undertaken to validate galectin\u20109 and CXCL<\/jats:styled-content>10 as biomarkers for disease activity in juvenile DM<\/jats:styled-content>, and to assess their disease specificity and potency in predicting the occurrence of flares.<\/jats:p><\/jats:sec>Methods<\/jats:title>Levels of galectin\u20109 and CXCL<\/jats:styled-content>10 were measured by multiplex immunoassay in serum samples from 125 unique patients with juvenile DM<\/jats:styled-content> in 3 international cross\u2010sectional cohorts and a local longitudinal cohort. The disease specificity of both proteins was examined in 50 adult patients with DM<\/jats:styled-content> or nonspecific myositis (NSM) and 61 patients with other systemic autoimmune diseases.<\/jats:p><\/jats:sec>Results<\/jats:title>Both cross\u2010sectionally and longitudinally, galectin\u20109 and CXCL<\/jats:styled-content>10 outperformed the currently used laboratory marker, creatine kinase (CK<\/jats:styled-content>), in distinguishing between juvenile DM<\/jats:styled-content> patients with active disease and those in remission (area under the receiver operating characteristic curve [AUC<\/jats:styled-content>] 0.86\u20130.90 for galectin\u20109 and CXCL<\/jats:styled-content>10; AUC<\/jats:styled-content> 0.66\u20130.68 for CK<\/jats:styled-content>). The sensitivity and specificity for active disease in juvenile DM<\/jats:styled-content> was 0.84 and 0.92, respectively, for galectin\u20109 and 0.87 and 1.00, respectively, for CXCL<\/jats:styled-content>10. In 10 patients with juvenile DM<\/jats:styled-content> who experienced a flare and were prospectively followed up, continuously elevated or rising biomarker levels suggested an imminent flare up to several months before the onset of symptoms, even in the absence of elevated CK<\/jats:styled-content> levels. Galectin\u20109 and CXCL<\/jats:styled-content>10 distinguished between active disease and remission in adult patients with DM<\/jats:styled-content> or NSM (P<\/jats:italic> = 0.0126 for galectin\u20109 and P<\/jats:italic> < 0.0001 for CXCL10) and were suited for measurement in minimally invasive dried blood spots (healthy controls versus juvenile DM, P<\/jats:italic> = 0.0040 for galectin\u20109 and P<\/jats:italic> < 0.0001 for CXCL10).<\/jats:p><\/jats:sec>Conclusion<\/jats:title>In this study, galectin\u20109 and CXCL<\/jats:styled-content>10 were validated as sensitive and reliable biomarkers for disease activity in juvenile DM<\/jats:styled-content>. Implementation of these biomarkers into clinical practice as tools to monitor disease activity and guide treatment might facilitate personalized treatment strategies.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.40881","type":"journal-article","created":{"date-parts":[[2019,3,12]],"date-time":"2019-03-12T18:13:34Z","timestamp":1552414414000},"page":"1377-1390","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":87,"title":["Galectin\u20109 and CXCL10 as Biomarkers for Disease Activity in Juvenile Dermatomyositis: A Longitudinal Cohort Study and Multicohort Validation"],"prefix":"10.1002","volume":"71","author":[{"given":"Judith","family":"Wienke","sequence":"first","affiliation":[{"name":"University Medical Centre Utrecht Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Felicitas","family":"Bellutti Enders","sequence":"additional","affiliation":[{"name":"Lausanne University Hospital, Lausanne, Switzerland, and University Hospital Basel Basel Switzerland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7565-1954","authenticated-orcid":false,"given":"Johan","family":"Lim","sequence":"additional","affiliation":[{"name":"Academic Medical Centre Amsterdam Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorre S.","family":"Mertens","sequence":"additional","affiliation":[{"name":"University Medical Centre Utrecht, Utrecht, The Netherlands, and Radboud University Medical Centre Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luuk L.","family":"van den Hoogen","sequence":"additional","affiliation":[{"name":"University Medical Centre Utrecht Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Camiel A.","family":"Wijngaarde","sequence":"additional","affiliation":[{"name":"University Medical Centre Utrecht Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joo Guan","family":"Yeo","sequence":"additional","affiliation":[{"name":"KK Women\u2019s and Children\u2019s Hospital, Duke\u2010NUS Medical School SingHealth Duke\u2010NUS Academic Medical Center Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alain","family":"Meyer","sequence":"additional","affiliation":[{"name":"CHU de Strasbourg Strasbourg France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henny G.","family":"Otten","sequence":"additional","affiliation":[{"name":"University Medical Centre Utrecht Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ruth D. 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