{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,17]],"date-time":"2026-07-17T03:44:55Z","timestamp":1784259895972,"version":"3.55.0"},"reference-count":21,"publisher":"Oxford University Press (OUP)","issue":"4","license":[{"start":{"date-parts":[[2025,1,17]],"date-time":"2025-01-17T00:00:00Z","timestamp":1737072000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,7,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:sec>\n                  <jats:title>Objective<\/jats:title>\n                  <jats:p>To investigate differences in autoantibodies, clinical features, and long-term outcomes between juvenile- and adult-onset systemic sclerosis (SSc).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Methods<\/jats:title>\n                  <jats:p>Autoantibodies and survival rates over a maximum of 20\u2009years were retrospectively analysed in 504 Japanese patients with SSc (juvenile-onset SSc, n\u2009=\u200917; adult-onset SSc, n\u2009=\u2009487) using data from Kyoto University Registry.<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Results<\/jats:title>\n                  <jats:p>: The autoantibodies observed were anti-topoisomerase-I (71% vs. 26%), anti-centromere (24% vs. 54%), and anti-RNA-polymerase-III (0% vs. 12%). A diffuse type and multiorgan involvement were observed in patients with anti-topoisomerase-I in both juvenile- and adult-onset SSc. In patients with anti-centromere, a diffuse type (juvenile-onset SSc vs. adult-onset SSc, 75% vs. 28%) and pulmonary fibrosis (50% vs. 17%) were more frequently observed in juvenile-onset SSc than in adult-onset SSc. Cox proportional hazard analyses showed that older onset (hazard ratio: 1.06, 95% confidence interval: 1.03\u20131.09) was associated with death, while autoantibodies were not significantly associated with death. Cumulative survival rates for 20\u2009years were similar between juvenile- and adult-onset SSc when classified based on the presence of anti-centromere (100% vs. 89%, P\u2009=\u2009.20) and anti-topoisomerase-I (90% vs. 90%, P\u2009=\u2009.70).<\/jats:p>\n               <\/jats:sec>\n               <jats:sec>\n                  <jats:title>Conclusions<\/jats:title>\n                  <jats:p>: Juvenile-onset SSc had more frequent diffuse-type and anti-topoisomerase-I. An older onset was slightly associated with mortality, whereas autoantibodies were not associated with mortality.<\/jats:p>\n               <\/jats:sec>","DOI":"10.1093\/mr\/roaf005","type":"journal-article","created":{"date-parts":[[2025,1,9]],"date-time":"2025-01-09T23:20:05Z","timestamp":1736464805000},"page":"683-690","source":"Crossref","is-referenced-by-count":2,"title":["Differences in the autoantibody phenotypes and long-term outcomes between juvenile- and adult-onset systemic sclerosis"],"prefix":"10.1093","volume":"35","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2521-246X","authenticated-orcid":false,"given":"Hideaki","family":"Tsuji","sequence":"first","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine , Kyoto,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1135-0463","authenticated-orcid":false,"given":"Mirei","family":"Shirakashi","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine , Kyoto,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6968-5712","authenticated-orcid":false,"given":"Ryosuke","family":"Hiwa","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine , Kyoto,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shuji","family":"Akizuki","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine , Kyoto,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ran","family":"Nakashima","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine , Kyoto,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akira","family":"Onishi","sequence":"additional","affiliation":[{"name":"Department of Advanced Medicine for Rheumatic Diseases, Kyoto University Graduate School of Medicine , Kyoto,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7082-4900","authenticated-orcid":false,"given":"Hajime","family":"Yoshifuji","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, Kyoto University Graduate School of Medicine , 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