{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T11:20:18Z","timestamp":1789816818280,"version":"4.0.1"},"reference-count":27,"publisher":"Oxford University Press (OUP)","issue":"2","license":[{"start":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T00:00:00Z","timestamp":1759968000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"funder":[{"name":"Japan Society for the Promotion of Science KAKENHI Grants-in-Aid for Scientific Research","award":["JP24K02230"],"award-info":[{"award-number":["JP24K02230"]}]},{"name":"Japan Society for the Promotion of Science KAKENHI Grants-in-Aid for Scientific Research","award":["JP23K15353"],"award-info":[{"award-number":["JP23K15353"]}]},{"name":"Japan Society for the Promotion of Science KAKENHI Grants-in-Aid for Scientific Research","award":["JP23K07229"],"award-info":[{"award-number":["JP23K07229"]}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP24ek0109677"],"award-info":[{"award-number":["JP24ek0109677"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP24ek0109648"],"award-info":[{"award-number":["JP24ek0109648"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP24ek0109617"],"award-info":[{"award-number":["JP24ek0109617"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP24ek0109760"],"award-info":[{"award-number":["JP24ek0109760"]}],"id":[{"id":"10.13039\/100009619","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100009619","name":"Japan Agency for Medical Research and Development","doi-asserted-by":"publisher","award":["JP24ek0109674"],"award-info":[{"award-number":["JP24ek0109674"]}],"id":[{"id":"10.13039\/100009619","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>VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an autoinflammatory disease caused by somatic variants of the UBA1 gene. Due to uncertainty around treatment strategies and limited therapeutic options, this study aimed to characterise the clinical treatment and outcomes of VEXAS syndrome patients in Japan.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We retrospectively analysed data on clinical manifestations, treatments, and outcomes from 46 male patients diagnosed with VEXAS syndrome. Twelve patients at our institution were evaluated for remission using the French VEXAS group criteria.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>All patients (median age at onset: 71.4\u00a0years) received systemic glucocorticoid therapy (mean maximum dose: 47.4\u00a0mg\/day; mean minimum dose: 8.5\u00a0mg\/day). Most patients required continuous glucocorticoid treatment of \u226510\u00a0mg\/day, and tapering was generally difficult. Among the patients followed at our institution, only 42% (5\/12) achieved complete remission at least once during follow-up. Tocilizumab was the most frequently administered immunosuppressant (n\u00a0=\u200924, 52.2%); Janus kinase inhibitors and azacitidine, reported to be effective overseas, were prescribed in only three and two cases, respectively.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>This study highlights the difficulty of treating patients with VEXAS syndrome in Japan. The development of more effective treatments is urgently needed to reduce glucocorticoid dependence and improve patient outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/mr\/roaf095","type":"journal-article","created":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T14:41:17Z","timestamp":1760020877000},"page":"307-312","source":"Crossref","is-referenced-by-count":6,"title":["High glucocorticoid dependency and limited therapeutic response in Japanese patients with VEXAS syndrome: a multicentre retrospective study"],"prefix":"10.1093","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1102-0475","authenticated-orcid":false,"given":"Ayaka","family":"Maeda","sequence":"first","affiliation":[{"name":"Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine , Yokohama ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9488-661X","authenticated-orcid":false,"given":"Yohei","family":"Kirino","sequence":"additional","affiliation":[{"name":"Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine , Yokohama ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7203-884X","authenticated-orcid":false,"given":"Naomi","family":"Tsuchida","sequence":"additional","affiliation":[{"name":"Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine , Yokohama ,","place":["Japan"]},{"name":"Department of Human Genetics, Yokohama City University Graduate School of Medicine , Yokohama ,","place":["Japan"]},{"name":"Department of Rare Disease Genomics, Yokohama City University Hospital , Yokohama ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4540-5277","authenticated-orcid":false,"given":"Yuri","family":"Uchiyama","sequence":"additional","affiliation":[{"name":"Department of Human Genetics, Yokohama City University Graduate School of Medicine , Yokohama ,","place":["Japan"]},{"name":"Department of Rare Disease Genomics, Yokohama City University Hospital , Yokohama ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0384-8678","authenticated-orcid":false,"given":"Daisuke","family":"Kobayashi","sequence":"additional","affiliation":[{"name":"Division of Clinical Nephrology and Rheumatology, Niigata University Graduate School of Medical and Dental Sciences , Niigata ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-6538-1881","authenticated-orcid":false,"given":"Tomoyuki","family":"Asano","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Fukushima Medical University School of Medicine , Fukushima ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6948-0338","authenticated-orcid":false,"given":"Yuji","family":"Ito","sequence":"additional","affiliation":[{"name":"Department of General Internal Medicine, Chutoen General Medical Center , Kakegawa ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9585-5885","authenticated-orcid":false,"given":"Kosuke","family":"Katsuo","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Kurashiki Central Hospital , Kurashiki ,","place":["Japan"]},{"name":"Department of Dermatology, Graduate School of Medicine, Kyoto University , Kyoto ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-2148-8475","authenticated-orcid":false,"given":"Ayaka","family":"Kato","sequence":"additional","affiliation":[{"name":"Department of General Internal Medicine, Gifu University Hospital , Gifu ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5237-2445","authenticated-orcid":false,"given":"Ichiro","family":"Mori","sequence":"additional","affiliation":[{"name":"Department of General Internal Medicine, Gifu University Hospital , Gifu ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9835-8040","authenticated-orcid":false,"given":"Hiroyuki","family":"Yamaguchi","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Teikyo University Chiba Medical Center , Ichihara ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0036-2039","authenticated-orcid":false,"given":"Toshimasa","family":"Shimizu","sequence":"additional","affiliation":[{"name":"Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences , Nagasaki ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Satoshi","family":"Kobayashi","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Rheumatology, Kyorin University School of Medicine , Mitaka ,","place":["Japan"]},{"name":"Department of Rheumatology, Hinoekimae Hospital , Hino ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4007-1589","authenticated-orcid":false,"given":"Mitsumasa","family":"Kishimoto","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Rheumatology, Kyorin University School of Medicine , Mitaka ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3967-6414","authenticated-orcid":false,"given":"Nobuhiro","family":"Oda","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Kameda Medical Center , Kamogawa ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ryo","family":"Rokutanda","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Department of Internal Medicine, Kameda Medical Center , Kamogawa ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5233-8308","authenticated-orcid":false,"given":"Ken","family":"Nagahata","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Clinical Immunology, Sapporo Medical University School of Medicine , Sapporo ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroshi","family":"Fujiwara","sequence":"additional","affiliation":[{"name":"Division of Rheumatology and Allergy, Osaka General Medical Center , Osaka ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0748-3784","authenticated-orcid":false,"given":"Hiroshi","family":"Kobayashi","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Tokyo Medical University Hachioji Medical Center , Hachioji ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7031-0651","authenticated-orcid":false,"given":"Yoichi","family":"Takeuchi","sequence":"additional","affiliation":[{"name":"Department of Nephrology and Rheumatology, Gunma University Graduate School of Medicine , Maebashi ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0002-2481-1169","authenticated-orcid":false,"given":"Hiroyuki","family":"Hagiyama","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Yokohama City Minato Red Cross Hospital , Yokohama ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Naoki","family":"Umeda","sequence":"additional","affiliation":[{"name":"Department of Dermatology, JA Hiroshima General Hospital , Hatsukaichi ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2983-2129","authenticated-orcid":false,"given":"Kaori","family":"Uchino","sequence":"additional","affiliation":[{"name":"Division of Hematology, Department of Internal Medicine, Aichi Medical University , Nagakute ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8724-6393","authenticated-orcid":false,"given":"Yuji","family":"Miyoshi","sequence":"additional","affiliation":[{"name":"Department of Rheumatic Diseases, Tokyo Metropolitan Tama Medical Center , Fuchu ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yukiko","family":"Hidaka","sequence":"additional","affiliation":[{"name":"Division of Respirology, Neurology and Rheumatology, Department of Medicine, Kurume University School of Medicine , Kurume ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0295-0301","authenticated-orcid":false,"given":"Hiroaki","family":"Ida","sequence":"additional","affiliation":[{"name":"Division of Respirology, Neurology and Rheumatology, Department of Medicine, Kurume University School of Medicine , Kurume ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Keiichi","family":"Akasaka","sequence":"additional","affiliation":[{"name":"Department of Respiratory Medicine, Saitama Red Cross Hospital , Saitama ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-8132-2224","authenticated-orcid":false,"given":"Kaori","family":"Kashino","sequence":"additional","affiliation":[{"name":"Department of Dermatology, Onomichi Municipal Hospital , Onomichi ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0774-0509","authenticated-orcid":false,"given":"Yusuke","family":"Yoshida","sequence":"additional","affiliation":[{"name":"Department of Clinical Immunology and Rheumatology, Hiroshima University Hospital , Hiroshima ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2890-0305","authenticated-orcid":false,"given":"Hidenori","family":"Ohnishi","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Graduate School of Medicine, Gifu University , Gifu ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1982-7019","authenticated-orcid":false,"given":"Noriyuki","family":"Takahashi","sequence":"additional","affiliation":[{"name":"Department of Education for Community-Oriented Medicine, Nagoya University Graduate School of Medicine , Nagoya ,","place":["Japan"]},{"name":"Department of General Medicine, Nagoya University Hospital , Nagoya ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4705-341X","authenticated-orcid":false,"given":"Yuichiro","family":"Fujieda","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Endocrinology and Nephrology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University , Sapporo ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8200-0340","authenticated-orcid":false,"given":"Nobuyuki","family":"Horita","sequence":"additional","affiliation":[{"name":"Chemotherapy Center, Yokohama City University Hospital , Yokohama ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0025-2617","authenticated-orcid":false,"given":"Kaoru","family":"Takase-Minegishi","sequence":"additional","affiliation":[{"name":"Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine , Yokohama ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9846-6500","authenticated-orcid":false,"given":"Naomichi","family":"Matsumoto","sequence":"additional","affiliation":[{"name":"Department of Human Genetics, Yokohama City University Graduate School of Medicine , Yokohama ,","place":["Japan"]},{"name":"Department of Rare Disease Genomics, Yokohama City University Hospital , Yokohama ,","place":["Japan"]},{"name":"Department of Clinical Genetics, Yokohama City University Hospital , Yokohama ,","place":["Japan"]},{"name":"Medical Genome Center, National Center of Neurology and Psychiatry , Kodaira ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8967-4954","authenticated-orcid":false,"given":"Hideaki","family":"Nakajima","sequence":"additional","affiliation":[{"name":"Department of Stem Cell and Immune Regulation, Yokohama City University Graduate School of Medicine , Yokohama ,","place":["Japan"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2025,10,9]]},"reference":[{"key":"2026030508170021100_ref1","doi-asserted-by":"crossref","first-page":"2628","DOI":"10.1056\/NEJMoa2026834","article-title":"Somatic mutations in UBA1 and severe adult-onset autoinflammatory disease","volume":"383","author":"Beck","year":"2020","journal-title":"N Engl J Med"},{"key":"2026030508170021100_ref2","doi-asserted-by":"crossref","DOI":"10.1093\/rheumatology\/keae530","article-title":"Low remission rates and high incidence of adverse events in a prospective VEXAS syndrome registry","volume":"64","author":"Kirino","year":"2025","journal-title":"Rheumatology (Oxford)"},{"key":"2026030508170021100_ref3","doi-asserted-by":"crossref","first-page":"1358","DOI":"10.1136\/ard-2024-225640","article-title":"Efficacy and safety of targeted therapies in VEXAS syndrome: retrospective study from the FRENVEX","volume":"83","author":"Hadjadj","year":"2024","journal-title":"Ann Rheum Dis"},{"key":"2026030508170021100_ref4","doi-asserted-by":"crossref","first-page":"1496","DOI":"10.1182\/blood.2022016985","article-title":"Translation of cytoplasmic UBA1 contributes to VEXAS syndrome pathogenesis","volume":"140","author":"Ferrada","year":"2022","journal-title":"Blood."},{"key":"2026030508170021100_ref5","doi-asserted-by":"crossref","first-page":"1444","DOI":"10.1182\/bloodadvances.2023012478","article-title":"Allogeneic hematopoietic cell transplantation for VEXAS syndrome: results of a multicenter study of the EBMT","volume":"8","author":"Gurnari","year":"2024","journal-title":"Blood Adv"},{"key":"2026030508170021100_ref6","doi-asserted-by":"crossref","first-page":"3518","DOI":"10.1093\/rheumatology\/kead240","article-title":"How to treat VEXAS syndrome: a systematic review on effectiveness and safety of current treatment strategies","volume":"62","author":"Boyadzhieva","year":"2023","journal-title":"Rheumatology (Oxford)"},{"key":"2026030508170021100_ref7","doi-asserted-by":"crossref","first-page":"1877","DOI":"10.1111\/j.1538-7836.2011.04443.x","article-title":"Racial differences in venous thromboembolism","volume":"9","author":"Zakai","year":"2011","journal-title":"J Thromb Haemost"},{"key":"2026030508170021100_ref8","doi-asserted-by":"crossref","first-page":"2056","DOI":"10.1093\/rheumatology\/kead425","article-title":"Efficient detection of somatic UBA1 variants and clinical scoring system predicting patients with variants in VEXAS syndrome","volume":"63","author":"Maeda","year":"2024","journal-title":"Rheumatology (Oxford)"},{"key":"2026030508170021100_ref9","doi-asserted-by":"crossref","first-page":"1057","DOI":"10.1136\/annrheumdis-2021-220089","article-title":"Pathogenic UBA1 variants associated with VEXAS syndrome in Japanese patients with relapsing polychondritis","volume":"80","author":"Tsuchida","year":"2021","journal-title":"Ann Rheum Dis"},{"key":"2026030508170021100_ref10","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1093\/mrcr\/rxad041","article-title":"Long-term remission of VEXAS syndrome achieved by a single course of CHOP therapy: a case report","volume":"8","author":"Miyoshi","year":"2023","journal-title":"Mod Rheumatol Case Rep"},{"key":"2026030508170021100_ref11","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1093\/mrcr\/rxac082","article-title":"Acute heart failure due to left common iliac arteriovenous fistula: a case of VEXAS 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