{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,28]],"date-time":"2025-10-28T10:02:58Z","timestamp":1761645778384,"version":"build-2065373602"},"reference-count":20,"publisher":"BMJ","issue":"10","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["BMJ Case Rep"],"accepted":{"date-parts":[[2025,10,6]]},"published-print":{"date-parts":[[2025,10]]},"abstract":"<jats:p>\n                    VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory and somatic) is a recently recognised, adult-onset autoinflammatory condition caused by somatic\n                    <jats:italic>UBA1<\/jats:italic>\n                    mutations, predominantly affecting older men. This condition often mimics autoimmune or infectious diseases, presenting with systemic inflammation and haematologic abnormalities. We describe a man in his 70s with seropositive rheumatoid arthritis who presented with a tender neck mass, recurrent fevers and elevated inflammatory markers. Symptoms improved with corticosteroids but relapsed on tapering. A bone marrow biopsy to investigate persistent inflammation revealed subtle cytoplasmic vacuolation in myeloid precursors, prompting targeted genetic testing that confirmed a somatic\n                    <jats:italic>UBA1<\/jats:italic>\n                    mutation. This case underscores the diagnostic complexity of VEXAS and the therapeutic challenges of managing this recently recognised entity.\n                  <\/jats:p>","DOI":"10.1136\/bcr-2025-268041","type":"journal-article","created":{"date-parts":[[2025,10,28]],"date-time":"2025-10-28T09:55:18Z","timestamp":1761645318000},"page":"e268041","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":0,"title":["VEXAS syndrome in a patient with seropositive rheumatoid arthritis: lessons in diagnostic vigilance"],"prefix":"10.1136","volume":"18","author":[{"given":"Stanley","family":"Du Preez","sequence":"first","affiliation":[{"name":"Department of Medicine, Redland Hospital, Cleveland, Queensland, Australia"},{"name":"School of Medicine, Griffith University Faculty of Health, Gold Coast, Queensland, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel","family":"Schuster","sequence":"additional","affiliation":[{"name":"Department of Immunology, Princess Alexandra Hospital, Woolloongabba, Queensland, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katrina","family":"Chakradeo","sequence":"additional","affiliation":[{"name":"Department of Medicine, Redland Hospital, Cleveland, Queensland, Australia"},{"name":"Frazer Institute, The University of Queensland, Brisbane, Queensland, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2025,10,28]]},"reference":[{"key":"2025102802551126000_18.10.e268041.1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa2026834"},{"key":"2025102802551126000_18.10.e268041.2","doi-asserted-by":"publisher","DOI":"10.4161\/cc.19978"},{"key":"2025102802551126000_18.10.e268041.3","doi-asserted-by":"crossref","first-page":"2694","DOI":"10.1093\/rheumatology\/keae123","article-title":"Comparing clinical features between males and females with VEXAS syndrome: data from literature analysis of patient reports","volume":"63","author":"Echerbault","year":"2024","journal-title":"Rheumatology (Oxford)"},{"key":"2025102802551126000_18.10.e268041.4","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1053\/j.seminhematol.2021.10.002","article-title":"VEXAS within the spectrum of rheumatologic disease","volume":"58","author":"Koster","year":"2021","journal-title":"Semin 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Immunol"},{"key":"2025102802551126000_18.10.e268041.13","doi-asserted-by":"crossref","DOI":"10.3389\/fimmu.2024.1466720","article-title":"Expanding the VEXAS diagnostic workup: the role of peripheral blood cytological analysis","volume":"15","author":"Baggio","year":"2024","journal-title":"Front Immunol"},{"key":"2025102802551126000_18.10.e268041.14","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1007\/s11912-023-01382-9","article-title":"Clonal Hematopoiesis of Indeterminate Potential: Current Understanding and Future Directions","volume":"25","author":"Singh","year":"2023","journal-title":"Curr Oncol Rep"},{"key":"2025102802551126000_18.10.e268041.15","doi-asserted-by":"publisher","DOI":"10.1182\/blood-2023-180395"},{"key":"2025102802551126000_18.10.e268041.16","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1080\/17474086.2023.2209715","article-title":"VEXAS: is it time to reshape the nosology of clonal hematopoiesis?","volume":"16","author":"Sujobert","year":"2023","journal-title":"Expert Rev Hematol"},{"key":"2025102802551126000_18.10.e268041.17","doi-asserted-by":"crossref","first-page":"1748","DOI":"10.1038\/s41433-024-03014-3","article-title":"Ocular and orbital manifestations in VEXAS syndrome","volume":"38","author":"Abumanhal","year":"2024","journal-title":"Eye (Lond)"},{"key":"2025102802551126000_18.10.e268041.18","doi-asserted-by":"crossref","first-page":"3682","DOI":"10.1182\/blood.2020010177","article-title":"Therapeutic options in VEXAS syndrome: insights from a retrospective series","volume":"137","author":"Bourbon","year":"2021","journal-title":"Blood"},{"key":"2025102802551126000_18.10.e268041.19","doi-asserted-by":"publisher","DOI":"10.1182\/blood.2022016642"},{"key":"2025102802551126000_18.10.e268041.20","doi-asserted-by":"crossref","DOI":"10.1186\/s13045-023-01471-z","article-title":"Ten years of treatment with ruxolitinib for myelofibrosis: a review of 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