{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,3]],"date-time":"2026-06-03T00:59:27Z","timestamp":1780448367711,"version":"3.54.1"},"reference-count":29,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2025,10,8]],"date-time":"2025-10-08T00:00:00Z","timestamp":1759881600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/pages\/standard-publication-reuse-rights"}],"funder":[{"DOI":"10.13039\/501100003478","name":"Japanese Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","award":["21FE1003"],"award-info":[{"award-number":["21FE1003"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,3,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>This study aimed to evaluate the diagnostic performance of the 2019 European League Against Rheumatism\/American College of Rheumatology (EULAR\/ACR-2019) classification criteria of systemic lupus erythematosus (SLE) and to clarify the clinical characteristics of Japanese childhood-onset SLE (cSLE).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We retrospectively analyzed clinical data registered in the Paediatric Rheumatology International Collaboration Unit Registry (PRICURE) version 2 up to March 31, 2023. Frequencies of individual items within the EULAR\/ACR-2019 criteria were compared with those observed in a Japanese adult SLE cohort.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>A total of 105 patients with cSLE, 19 with Juvenile dermatomyositis (JDM), 27 with primary Sj\u00f6gren\u2019s disease (pSjD), and 9 with mixed connective-tissue disease (MCTD) were included. The sensitivity of the EULAR\/ACR-2019 criteria was 97.1%. The specificity was 94.7% for JDM, 92.6% for pSjD, 55.6% for MCTD, and 87.3% for all disease controls. cSLE patients in this cohort more frequently exhibited renal involvement, low serum C3 or C4 levels, and positivity for antiphospholipid and anti-double-stranded DNA antibodies, but joint symptoms were less common than in adult SLE patients.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>Although the EULAR\/ACR-2019 criteria are generally applicable, the limited specificity for MCTD necessitates careful differential diagnosis. Japanese cSLE is commonly characterized by renal involvement, hypocomplementemia, and SLE-related autoantibody positivity.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/mr\/roaf088","type":"journal-article","created":{"date-parts":[[2025,10,7]],"date-time":"2025-10-07T11:41:39Z","timestamp":1759837299000},"page":"239-245","source":"Crossref","is-referenced-by-count":1,"title":["Validation of the 2019 EULAR\/ACR classification criteria and clinical characteristics of childhood-onset Japanese patients with systemic lupus erythematosus"],"prefix":"10.1093","volume":"36","author":[{"given":"Hitoshi","family":"Irabu","sequence":"first","affiliation":[{"name":"Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Institute of Science Tokyo (ISCT) , Tokyo ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Asami","family":"Ohara","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Graduate School of Medicine, Yokohama City University , Yokohama ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aki","family":"Nakamura","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Kagoshima University Hospital , Kagoshima ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hidehiko","family":"Narazaki","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Nippon Medical School , Tokyo ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuko","family":"Hayashi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Perinatal and Maternal Medicine (Ibaraki), Graduate School of Medical and Dental Sciences, Institute of Science Tokyo (ISCT) , Tokyo ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuichi","family":"Yamasaki","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Kagoshima University Hospital , Kagoshima ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naomi","family":"Iwata","sequence":"additional","affiliation":[{"name":"Department of Infection and Immunology, Allergy and Immunology Center, Aichi Children\u2019s Health and Medical Center , Obu ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shiro","family":"Ohshima","sequence":"additional","affiliation":[{"name":"Department of Clinical Research, NHO Osaka Minami Medical Center , Osaka ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susumu","family":"Nishiyama","sequence":"additional","affiliation":[{"name":"Rheumatic Disease Center, Kurashiki Medical Center , Kurashiki ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasuo","family":"Nakagishi","sequence":"additional","affiliation":[{"name":"Department of Pediatric Rheumatology, Hyogo Prefectural Kobe Children\u2019s Hospital , Kobe ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daisuke","family":"Fukuhara","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Kyorin University School of Medicine , Tokyo ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroyuki","family":"Ishida","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Kyoto City Hospital , Kyoto ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Miwa","family":"Goto","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Faculty of Medicine, University of Yamanashi , Yamanashi ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kunio","family":"Hashimoto","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Nagasaki University Graduate School of Biomedical Sciences , Nagasaki ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryuhei","family":"Yasuoka","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Hamamatsu University School of Medicine , Shizuoka ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tomo","family":"Nozawa","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Graduate School of Medicine, Yokohama City University , Yokohama ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Takako","family":"Miyamae","sequence":"additional","affiliation":[{"name":"Department of Pediatric Rheumatology, Institute of Rheumatology, Tokyo Women\u2019s Medical University , Tokyo ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroaki","family":"Umebayashi","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Infectious Disease, Miyagi Children\u2019s Hospital , Sendai ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masaaki","family":"Mori","sequence":"additional","affiliation":[{"name":"Division of Rheumatology and Allergology, Department of Internal Medicine, St. Marianna University School of Medicine , Kawasaki ,","place":["Japan"]},{"name":"Joint Research Department of Lifelong Immunotherapy, Institute of Science Tokyo (ISCT) , Tokyo ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1077-7772","authenticated-orcid":false,"given":"Masaki","family":"Shimizu","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Perinatal and Maternal Medicine (Ibaraki), Graduate School of Medical and Dental Sciences, Institute of Science Tokyo (ISCT) , Tokyo 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