{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T07:50:55Z","timestamp":1781769055461,"version":"3.54.5"},"reference-count":15,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2026,3,6]],"date-time":"2026-03-06T00:00:00Z","timestamp":1772755200000},"content-version":"vor","delay-in-days":5,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,3,1]],"date-time":"2026-03-01T00:00:00Z","timestamp":1772323200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/100007253","name":"Nancy Taylor Foundation for Chronic Disease","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007253","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":[[2026,3]]},"abstract":"\n Objective<\/jats:title>\n Juvenile\u2010onset systemic sclerosis (jSSc) is a rare, heterogeneous pediatric autoimmune disease. Existing severity tools are often adapted from adult systemic sclerosis and lack pediatric\u2010specific validation. The Juvenile Systemic Sclerosis Severity Score (J4S) was developed to address this gap by capturing multiorgan disease burden. This study evaluated the clinical use of the J4S using data from the National Registry for Childhood Onset Scleroderma (NRCOS).<\/jats:p>\n <\/jats:sec>\n \n Methods<\/jats:title>\n We applied the J4S to 58 patients in the NRCOS cohort, analyzing scores at baseline and at one\u2010, two\u2010, and three\u2010year follow\u2010ups. Subdomain scores were assessed to evaluate organ\u2010specific trends. An exploratory version with equal domain weighting was also tested for sensitivity.<\/jats:p>\n <\/jats:sec>\n \n Results<\/jats:title>\n \n At baseline, the mean J4S was 8.18 (\u00b13.64), with respiratory involvement as the primary contributor. Although no significant change in total scores was observed at one (\n P<\/jats:italic>\n = 0.264) or two years (\n P<\/jats:italic>\n = 0.06), domain\u2010specific improvements were noted, particularly in the skin, vascular, gastrointestinal, and musculoskeletal systems. By three years, total scores declined significantly (\n P<\/jats:italic>\n = 0.028). Two patients experienced sharp score increases due to respiratory decline; one patient died during the study. The exploratory equal\u2010weight scoring approach improved sensitivity to change and identified significant declines at each time point.\n <\/jats:p>\n <\/jats:sec>\n \n Conclusion<\/jats:title>\n The J4S is a valuable longitudinal tool for tracking disease severity and organ involvement in jSSc. It can identify both stable and progressive disease and may help detect patients at risk for poor outcomes. Modifying domain weighting may enhance responsiveness. Future multicenter validation and clinical trial integration are warranted.<\/jats:p>\n <\/jats:sec>","DOI":"10.1002\/acr2.90008","type":"journal-article","created":{"date-parts":[[2026,3,8]],"date-time":"2026-03-08T16:43:37Z","timestamp":1772988217000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Longitudinal Assessment of the Juvenile Systemic Sclerosis Severity Score: Application in the National Registry for Childhood Onset Scleroderma"],"prefix":"10.1002","volume":"8","author":[{"given":"Samantha A.","family":"Branton","sequence":"first","affiliation":[{"name":"University of Pittsburgh Pittsburgh Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1662-143X","authenticated-orcid":false,"given":"Kathryn S.","family":"Torok","sequence":"additional","affiliation":[{"name":"University of Pittsburgh Pittsburgh Pennsylvania"},{"name":"Department of Pediatrics, Division of Pediatric Rheumatology University of Pittsburgh Medical Center Children's Hospital of Pittsburgh Pittsburgh Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2026,3,6]]},"reference":[{"issue":"7","key":"e_1_2_7_2_1","first-page":"1573","article-title":"Criteria for the classification of early systemic sclerosis","volume":"28","author":"LeRoy EC","year":"2001","journal-title":"J Rheumatol"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.38098"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.20070"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/1756-185X.13716"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.23547"},{"issue":"5","key":"e_1_2_7_7_1","first-page":"1004","article-title":"Childhood onset systemic sclerosis: classification, clinical and serologic features, and survival in comparison with adult onset disease","volume":"33","author":"Scalapino K","year":"2006","journal-title":"J Rheumatol"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.22207"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.24609"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1186\/s12969-024-01043-6"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1038\/nrrheum.2011.148"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.34652"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.20035"},{"issue":"3","key":"e_1_2_7_14_1","first-page":"S42","article-title":"Assessment of disease severity and prognosis","volume":"21","author":"Medsger TA","year":"2003","journal-title":"Clin Exp Rheumatol"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.24499"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.1080\/1744666X.2023.2298354"}],"container-title":["ACR Open Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/acr2.90008","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/acr2.90008","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/acr2.90008","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,11]],"date-time":"2026-04-11T06:56:33Z","timestamp":1775890593000},"score":1,"resource":{"primary":{"URL":"https:\/\/acrjournals.onlinelibrary.wiley.com\/doi\/10.1002\/acr2.90008"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,3]]},"references-count":15,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2026,3]]}},"alternative-id":["10.1002\/acr2.90008"],"URL":"https:\/\/doi.org\/10.1002\/acr2.90008","archive":["Portico"],"relation":{},"ISSN":["2578-5745","2578-5745"],"issn-type":[{"value":"2578-5745","type":"print"},{"value":"2578-5745","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,3]]},"assertion":[{"value":"2025-08-02","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2025-12-02","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2026-03-06","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e90008"}}