{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T06:59:39Z","timestamp":1780469979814,"version":"3.54.1"},"reference-count":47,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2024,2,15]],"date-time":"2024-02-15T00:00:00Z","timestamp":1707955200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000980","name":"AF","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000980","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000069","name":"NIH National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100012264","name":"NIH","doi-asserted-by":"publisher","award":["RC2\u2010AR0\u201058934"],"award-info":[{"award-number":["RC2\u2010AR0\u201058934"]}],"id":[{"id":"10.13039\/501100012264","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006513","name":"DCRI","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006513","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis Care &amp;amp; Research"],"published-print":{"date-parts":[[2024,5]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>Juvenile localized scleroderma (jLS) is a chronic autoimmune disease commonly associated with poor outcomes, including contractures, hemiatrophy, uveitis, and seizures. Despite improvements in treatment, &gt;25% of patients with jLS have functional impairment. To improve patient evaluation, our workgroup developed the Localized scleroderma Total Severity Scale (LoTSS), an overall disease severity measure.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>LoTSS was developed as a weighted measure by a consensus process involving literature review, surveys, case vignettes, and multicriteria decision analysis. Feasibility was assessed in larger Childhood Arthritis and Rheumatology Research Alliance groups. Construct validity with physician assessment and inter\u2010rater reliability was assessed using case vignettes. Additional evaluation was performed in a prospective patient cohort initiating treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>LoTSS severity items were organized into modules that reflect jLS disease patterns, with modules for skin, extracutaneous, and craniofacial manifestations. Construct validity of LoTSS was supported by a strong positive correlation with the Physician Global Assessment (PGA) of severity and damage and weak positive correlation with PGA\u2010Activity, as expected. LoTSS was responsive, with a small effect size identified. Moderate\u2010to\u2010excellent inter\u2010rater reliability was demonstrated. LoTSS was able to discriminate between patient subsets, with higher scores identified in those with greater disease burden and functional limitation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>We developed a new LS measure for assessing cutaneous and extracutaneous severity and have shown it to be reliable, valid, and responsive. LoTSS is the first measure that assesses and scores all the major extracutaneous manifestations in LS. Our findings suggest LoTSS could aid assessment and management of patients and facilitate outcome evaluation in treatment studies.<\/jats:p><jats:p><jats:boxed-text content-type=\"graphic\" position=\"anchor\"><jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/acr25281-toc-0001-m.png\"><jats:alt-text>image<\/jats:alt-text><\/jats:graphic><\/jats:boxed-text><\/jats:p><\/jats:sec>","DOI":"10.1002\/acr.25281","type":"journal-article","created":{"date-parts":[[2023,12,27]],"date-time":"2023-12-27T00:40:10Z","timestamp":1703637610000},"page":"616-626","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Capturing the Range of Disease Involvement in Localized Scleroderma: The Localized Scleroderma Total Severity Scale"],"prefix":"10.1002","volume":"76","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8276-2866","authenticated-orcid":false,"given":"Suzanne C.","family":"Li","sequence":"first","affiliation":[{"name":"Joseph M. 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Egla","family":"Rabinovich","sequence":"additional","affiliation":[{"name":"Duke University School of Medicine  Durham North Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mara L.","family":"Becker","sequence":"additional","affiliation":[{"name":"Duke University School of Medicine  Durham North Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathryn S.","family":"Torok","sequence":"additional","affiliation":[{"name":"University of Pittsburgh  Pittsburgh Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Polly J.","family":"Ferguson","sequence":"additional","affiliation":[{"name":"University of Iowa Carver College of Medicine  Iowa City"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7516-0848","authenticated-orcid":false,"given":"Fatma","family":"Dedeoglu","sequence":"additional","affiliation":[{"name":"Boston Children's Hospital  Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sandy","family":"Hong","sequence":"additional","affiliation":[{"name":"University of Iowa Stead Family Children's Hospital  Iowa City Iowa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vidya","family":"Sivaraman","sequence":"additional","affiliation":[{"name":"The Ohio State University and Nationwide Children's Hospital  Columbus Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronald M.","family":"Laxer","sequence":"additional","affiliation":[{"name":"Temerty Faculty of Medicine, University of Toronto, and The Hospital for Sick Children  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katie","family":"Stewart","sequence":"additional","affiliation":[{"name":"Texas Children's Hospital and Baylor College of Medicine  Austin Texas"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria F.","family":"Ibarra","sequence":"additional","affiliation":[{"name":"Children's Mercy Hospital  Kansas City Missouri"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"suffix":"II","given":"Thomas","family":"Mason","sequence":"additional","affiliation":[{"name":"Mayo Clinic  Rochester Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gloria","family":"Higgins","sequence":"additional","affiliation":[{"name":"The Ohio State University and Nationwide Children's Hospital  Columbus Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Pope","sequence":"additional","affiliation":[{"name":"Temerty Faculty of Medicine, University of Toronto, and The Hospital for Sick Children  Toronto Ontario Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaohu","family":"Li","sequence":"additional","affiliation":[{"name":"Stevens Institute of Technology  Hoboken New Jersey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6478-5690","authenticated-orcid":false,"given":"Tara","family":"Lozy","sequence":"additional","affiliation":[{"name":"Center for Discovery and Innovation, Hackensack Meridian Health  Nutley New Jersey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert C.","family":"Fuhlbrigge","sequence":"additional","affiliation":[{"name":"University of Colorado and Colorado Children's Hospital  Aurora"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"for the CARRA Registry Investigators","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2024,2,15]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2018.02.004"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr2.1019"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/bjd.12606"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2021.01.068"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keab238"},{"issue":"2","key":"e_1_2_7_7_1","first-page":"171","article-title":"Localized scleroderma in adults and children. 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