{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,18]],"date-time":"2026-06-18T07:50:54Z","timestamp":1781769054219,"version":"3.54.5"},"reference-count":19,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2018,11,8]],"date-time":"2018-11-08T00:00:00Z","timestamp":1541635200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100007253","name":"Nancy Taylor Foundation for Chronic Diseases","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007253","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["RC2AR058934"],"award-info":[{"award-number":["RC2AR058934"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000980","name":"Arthritis Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000980","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000069","name":"National Institute of Arthritis and Musculoskeletal and Skin Diseases","doi-asserted-by":"publisher","award":["K 23 AR059722"],"award-info":[{"award-number":["K 23 AR059722"]}],"id":[{"id":"10.13039\/100000069","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis Care &amp; Research"],"published-print":{"date-parts":[[2018,12]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To investigate clinical manifestations of juvenile systemic sclerosis (<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c; scleroderma), including disease characteristics and patient quality of life, using the multinational Childhood Arthritis and Rheumatology Research Alliance (<jats:styled-content style=\"fixed-case\">CARRA<\/jats:styled-content>) Legacy Registry.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients with juvenile <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c were prospectively enrolled between 2010 and 2013. The diagnosis of juvenile <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c was determined by the enrolling pediatric rheumatologist, with the requirement for disease onset prior to age 18 years. Collected data included demographics, disease characteristics, medication exposure, and quality of life metrics.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>In total, 64 patients with juvenile <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c were enrolled a median of 3.6 years after disease onset, which occurred at a median age of 10.3 years. The most common organ manifestations were dermatologic and vascular, followed by musculoskeletal, gastrointestinal, and pulmonary; in 38% of patients, \u22654 organ systems were affected. Patients with juvenile <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c had significantly more disability at enrollment compared with <jats:styled-content style=\"fixed-case\">CARRA<\/jats:styled-content> Legacy Registry patients with juvenile idiopathic arthritis, dermatomyositis, or systemic lupus erythematosus. Although physician\u2010reported measures correlated most closely with arthritis, dermatologic manifestations, and pulmonary manifestations, poor patient\u2010reported measures were associated with gastrointestinal involvement. During &gt;50 person\u2010years of follow\u2010up, most organ manifestations remained stable, and no mortality or development of new solid organ involvement after enrollment was reported.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>In the first multicenter prospective cohort of patients with juvenile <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c in North America, the disease burden was high: multiorgan manifestations were common, and functional disability was greater than that observed in patients with other childhood\u2010onset rheumatic diseases. Gastrointestinal involvement had the greatest impact on quality of life.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr.23547","type":"journal-article","created":{"date-parts":[[2018,2,18]],"date-time":"2018-02-18T20:33:19Z","timestamp":1518985999000},"page":"1806-1813","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":45,"title":["Clinical Characteristics and Factors Associated With Disability and Impaired Quality of Life in Children With Juvenile Systemic Sclerosis: Results From the Childhood Arthritis and Rheumatology Research Alliance Legacy Registry"],"prefix":"10.1002","volume":"70","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6257-2301","authenticated-orcid":false,"given":"Brandi E.","family":"Stevens","sequence":"first","affiliation":[{"name":"Riley Hospital for Children at Indiana University Health Indianapolis, and University of Pittsburgh Medical Center Children's Hospital of Pittsburgh Pittsburgh Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathryn S.","family":"Torok","sequence":"additional","affiliation":[{"name":"University of Pittsburgh Medical Center Children's Hospital of Pittsburgh Pittsburgh Pennsylvania"}],"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 Meridian Health Hackensack New Jersey"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicole","family":"Hershey","sequence":"additional","affiliation":[{"name":"University of Pittsburgh Medical Center Children's Hospital of Pittsburgh Pittsburgh Pennsylvania"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Megan","family":"Curran","sequence":"additional","affiliation":[{"name":"Northwestern University Feinberg School of Medicine Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gloria C.","family":"Higgins","sequence":"additional","affiliation":[{"name":"Ohio State University and Nationwide Children's Hospital Columbus"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katharine F.","family":"Moore","sequence":"additional","affiliation":[{"name":"University of Colorado Children's Hospital Colorado Aurora"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Egla Rabinovich","sequence":"additional","affiliation":[{"name":"Duke University Medical Center Durham North Carolina"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samuel","family":"Dodson","sequence":"additional","affiliation":[{"name":"University of Washington Seattle"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anne M.","family":"Stevens","sequence":"additional","affiliation":[{"name":"University of Washington Seattle"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"and the Childhood Arthritis and Rheumatology Research Alliance Registry Investigators","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,11,8]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.20070"},{"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\/art.22551"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.22207"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kes144"},{"key":"e_1_2_7_7_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/39.5.556"},{"key":"e_1_2_7_8_1","first-page":"1004","article-title":"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_9_1","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.100001"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.22142"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780371209"},{"issue":"4","key":"e_1_2_7_12_1","first-page":"S1","article-title":"Cross\u2010cultural adaptation and psychometric evaluation of the Childhood Health Assessment Questionnaire (CHAQ) and the Child Health Questionnaire (CHQ) in 32 countries: review of the general methodology","volume":"19","author":"Ruperto N","year":"2001","journal-title":"Clin Exp Rheumatol"},{"key":"e_1_2_7_13_1","unstructured":"LandgrafJM AbetzL WareJE.Child health questionnaire (CHQ): a user's manual. 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