{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T07:18:46Z","timestamp":1780471126634,"version":"3.54.1"},"reference-count":19,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2019,4,10]],"date-time":"2019-04-10T00:00:00Z","timestamp":1554854400000},"content-version":"vor","delay-in-days":9,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["RC2AR058934"],"award-info":[{"award-number":["RC2AR058934"]}],"id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006513","name":"Duke Clinical Research Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006513","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["2T32AI007062\u201033"],"award-info":[{"award-number":["2T32AI007062\u201033"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["5T32HD060558\u201002"],"award-info":[{"award-number":["5T32HD060558\u201002"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["5T32AI007217\u201028"],"award-info":[{"award-number":["5T32AI007217\u201028"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["ACR Open Rheumatology"],"published-print":{"date-parts":[[2019,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>Localized scleroderma (<jats:styled-content style=\"fixed-case\">LS<\/jats:styled-content>) is a chronic inflammatory and fibrosing skin disorder. We present baseline data on the juvenile <jats:styled-content style=\"fixed-case\">LS<\/jats:styled-content> (<jats:styled-content style=\"fixed-case\">jLS<\/jats:styled-content>) cohort from the Childhood Arthritis and Rheumatology Research Alliance (<jats:styled-content style=\"fixed-case\">CARRA<\/jats:styled-content>) Legacy Registry, a multicenter observational registry of pediatric rheumatologic disorders.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This is a cross\u2010sectional analysis of children with <jats:styled-content style=\"fixed-case\">jLS<\/jats:styled-content> enrolled in the <jats:styled-content style=\"fixed-case\">CARRA<\/jats:styled-content> Legacy Registry between May 2010 and April 2014. Descriptive statistics were used for demographic, clinical, and laboratory features. Data analysis included two\u2010sample <jats:italic>t<\/jats:italic> test, \u03c7<jats:sup>2<\/jats:sup> test, Fisher's exact test, linear\/logistic regression, and analysis of variance.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of 381 children with <jats:styled-content style=\"fixed-case\">jLS<\/jats:styled-content>, 76% were female and 80% Caucasian. Mean onset age was 8.2 years, with 17% having a 2\u2010year or greater delay to first pediatric rheumatology (<jats:styled-content style=\"fixed-case\">PRH<\/jats:styled-content>) visit. Linear scleroderma was the most common subtype (54%). Antinuclear antibody (<jats:styled-content style=\"fixed-case\">ANA<\/jats:styled-content>) positivity was associated with joint contracture (<jats:italic>P<\/jats:italic> = 0.04), muscle atrophy (<jats:italic>P<\/jats:italic> = 0.014), and extremity shortening (<jats:italic>P<\/jats:italic> = 0.007). Elevated aldolase was associated with joint contracture (<jats:italic>P<\/jats:italic> = 0.008) and elevated creatine kinase (<jats:styled-content style=\"fixed-case\">CK<\/jats:styled-content>) with muscle atrophy (<jats:italic>P<\/jats:italic> = 0.028) and extremity shortening (<jats:italic>P<\/jats:italic> = 0.016). Children with functional limitation (27%) had earlier first <jats:styled-content style=\"fixed-case\">PRH<\/jats:styled-content> visit compared with those without (<jats:italic>P<\/jats:italic> = 0.01). Poorer function correlated with muscle atrophy, joint contracture, and extremity shortening (<jats:italic>P<\/jats:italic> &lt; 0.001). Methotrexate (97%) and corticosteroids (68%) were the most common medications used.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Children with <jats:styled-content style=\"fixed-case\">jLS<\/jats:styled-content> without joint limitation are referred later, highlighting the insidious onset and need for educating referring providers. Poorer function correlated with muscle atrophy, joint contracture, and limb shortening. <jats:styled-content style=\"fixed-case\">ANA<\/jats:styled-content> positivity and elevated <jats:styled-content style=\"fixed-case\">CK<\/jats:styled-content> or aldolase were associated with muscle atrophy, joint contracture, and\/or limb shortening, suggesting predictors of muscle involvement.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr2.1019","type":"journal-article","created":{"date-parts":[[2019,4,10]],"date-time":"2019-04-10T05:56:06Z","timestamp":1554875766000},"page":"119-124","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":44,"title":["Baseline Description of the Juvenile Localized Scleroderma Subgroup From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry"],"prefix":"10.1002","volume":"1","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6394-6843","authenticated-orcid":false,"given":"Eveline Y.","family":"Wu","sequence":"first","affiliation":[{"name":"University of North Carolina at Chapel Hill"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8276-2866","authenticated-orcid":false,"given":"Suzanne C.","family":"Li","sequence":"additional","affiliation":[{"name":"Hackensack University Medical Center  Hackensack New Jersey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathryn S.","family":"Torok","sequence":"additional","affiliation":[{"name":"Children\u2019s Hospital of Pittsburgh of UPMC  Pittsburgh Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yamini V.","family":"Virkud","sequence":"additional","affiliation":[{"name":"Massachusetts General Hospital for Children  Boston"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert C.","family":"Fuhlbrigge","sequence":"additional","affiliation":[{"name":"University of Colorado\u2010Denver  Aurora"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C. Egla","family":"Rabinovich","sequence":"additional","affiliation":[{"name":"Duke University Medical Center  Durham North Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry Investigators","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,4,10]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pcl.2012.03.011"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rdc.2013.05.003"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pcl.2018.04.002"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kei251"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/ker142"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/ker521"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2133.2011.10600.x"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.bor.0000245727.40630.c3"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rdc.2007.11.004"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.21264"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1001\/archdermatol.2010.239"},{"key":"e_1_2_8_13_1","first-page":"73","article-title":"The epidemiology of morphea (localized scleroderma) in Olmsted County 1960\u20101993","volume":"24","author":"Peterson LS","year":"1997","journal-title":"J Rheumatol"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.23101"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780350502"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11136-013-0581-8"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1001\/archdermatol.2009.79"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaad.2012.01.011"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamadermatol.2013.4207"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-018-3144-8"}],"container-title":["ACR Open Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Facr2.1019","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/acr2.1019","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/acr2.1019","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/acr2.1019","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,2]],"date-time":"2025-10-02T16:17:55Z","timestamp":1759421875000},"score":1,"resource":{"primary":{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/10.1002\/acr2.1019"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,4]]},"references-count":19,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2019,4]]}},"alternative-id":["10.1002\/acr2.1019"],"URL":"https:\/\/doi.org\/10.1002\/acr2.1019","archive":["Portico"],"relation":{},"ISSN":["2578-5745","2578-5745"],"issn-type":[{"value":"2578-5745","type":"print"},{"value":"2578-5745","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,4]]},"assertion":[{"value":"2019-04-10","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}