{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,15]],"date-time":"2026-06-15T17:17:29Z","timestamp":1781543849107,"version":"3.54.5"},"reference-count":23,"publisher":"BMJ","issue":"3","license":[{"start":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T00:00:00Z","timestamp":1759276800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2025,10,1]],"date-time":"2025-10-01T00:00:00Z","timestamp":1759276800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"funder":[{"DOI":"10.13039\/100001003","name":"boehringer ingelheim","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100001003","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Journal of Scleroderma and Related Disorders"],"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:sec>\n                    <jats:title>Objectives:<\/jats:title>\n                    <jats:p>Systemic sclerosis is a rare autoimmune connective tissue disease characterized by progressive skin and organ fibrosis, and diffuse fibro-proliferative vascular modifications. Roughly 2%\u201310% of systemic sclerosis cases develop during juvenile years (under 18\u2009years of age); however, there are limited data on the incidence and prevalence of juvenile systemic sclerosis. This analysis assessed the incidence and prevalence of juvenile systemic sclerosis in the United States, overall and by age group.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>\n                      Juvenile systemic sclerosis patients with \u2a7e2 medical diagnostic claims for systemic sclerosis within a 1-year period were identified from the Optum de-identified Clinformatics\n                      <jats:sup>\u00ae<\/jats:sup>\n                      Data Mart Database between 2007 and 2021, using International Classification of Diseases diagnostic codes. Incidence and prevalence rates were estimated in the overall population and in a subgroup of patients with at least one filled prescription or infusion of methotrexate, mycophenolate mofetil or cyclophosphamide. Estimates were sex- and\/or age-adjusted according to the 2020 US census.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>Forty-eight incident and 103 prevalent cases were identified. Overall, the age- and sex-adjusted incidence and prevalence of juvenile systemic sclerosis were 2.4 per million person-years and 12.0 per million, respectively. When stratified by age group, incidence and prevalence rates increased with age in the overall and subgroup analyses.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>In this analysis, incidence and prevalence rates of juvenile systemic sclerosis increased with age. Increasing incidence and prevalence were observed from 10 years old and from 6\u2009years old, respectively. While the overall data are broadly consistent with previous reports, these data provide new information on the estimated incidence and prevalence of juvenile systemic sclerosis by age group.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/23971983251370871","type":"journal-article","created":{"date-parts":[[2025,10,16]],"date-time":"2025-10-16T11:06:11Z","timestamp":1760612771000},"page":"303-310","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":1,"title":["Incidence and prevalence of juvenile SSc in the United States: An analysis by age group"],"prefix":"10.1136","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0009-0008-1281-5780","authenticated-orcid":false,"given":"John","family":"Mattson","sequence":"first","affiliation":[{"name":"Boehringer Ingelheim Pharmaceuticals Inc, Ridgefield, CT, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Haikun","family":"Bao","sequence":"additional","affiliation":[{"name":"Boehringer Ingelheim Pharmaceuticals Inc, Ridgefield, CT, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mary","family":"Flack","sequence":"additional","affiliation":[{"name":"Boehringer Ingelheim Pharmaceuticals Inc, Ridgefield, CT, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1325-8807","authenticated-orcid":false,"given":"Karen","family":"Coeytaux","sequence":"additional","affiliation":[{"name":"Boehringer Ingelheim Pharmaceuticals Inc, Ridgefield, CT, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2025,10,16]]},"reference":[{"key":"e_1_3_3_2_2","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2019.01352"},{"key":"e_1_3_3_3_2","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/kel292"},{"key":"e_1_3_3_4_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.clim.2023.109267"},{"key":"e_1_3_3_5_2","doi-asserted-by":"publisher","DOI":"10.1007\/s12016-021-08889-8"},{"key":"e_1_3_3_6_2","doi-asserted-by":"publisher","DOI":"10.1186\/1546-0096-5-6"},{"key":"e_1_3_3_7_2","doi-asserted-by":"publisher","DOI":"10.1002\/acr.23547"},{"key":"e_1_3_3_8_2","doi-asserted-by":"publisher","DOI":"10.1177\/2397198319899013"},{"key":"e_1_3_3_9_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10067-018-4182-3"},{"issue":"5","key":"e_1_3_3_10_2","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","unstructured":"Scalapino K, Arkachaisri T, Lucas M, et al. Childhood onset systemic sclerosis: classification, clinical and serologic features, and survival in comparison with adult onset disease. J Rheumatol 2006; 33(5): 1004\u20131013.","journal-title":"J Rheumatol"},{"key":"e_1_3_3_11_2","doi-asserted-by":"publisher","DOI":"10.1177\/2397198318763701"},{"key":"e_1_3_3_12_2","doi-asserted-by":"publisher","DOI":"10.1177\/2397198318769796"},{"key":"e_1_3_3_13_2","doi-asserted-by":"publisher","DOI":"10.1038\/nrrheum.2011.148"},{"key":"e_1_3_3_14_2","doi-asserted-by":"publisher","DOI":"10.1007\/s11926-009-0014-4"},{"key":"e_1_3_3_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.berh.2019.101428"},{"key":"e_1_3_3_16_2","unstructured":"Foeldvari I Klotsche J Kasapcopur O et al. POS0172 Diffuse juvenile systemic sclerosis patients show distinct organ involvement and have more severe disease in the largest jSSc cohort of the world. Results from the Juvenile Scleroderma Inception Cohort. www.juvenile-scleroderma.com. Ann Rheum Dis 2022; 81: 315\u2013316."},{"key":"e_1_3_3_17_2","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/ken388"},{"key":"e_1_3_3_18_2","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.100001"},{"key":"e_1_3_3_19_2","doi-asserted-by":"publisher","DOI":"10.1002\/acr.20070"},{"key":"e_1_3_3_20_2","doi-asserted-by":"publisher","DOI":"10.1177\/2397198319873526"},{"key":"e_1_3_3_21_2","unstructured":"US Census Bureau. 2020 demographic analysis estimates press kit https:\/\/www.census.gov\/newsroom\/press-kits\/2020\/2020-demographic-analysis.html (2022 accessed 19 June 2023)."},{"key":"e_1_3_3_22_2","doi-asserted-by":"crossref","unstructured":"Foeldvari I Klotsche J Kasapcopur O et al. SAT0479 update from the Juvenile Scleroderma Inception Cohort. www.juvenile-scleroderma.com. 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