{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T03:14:23Z","timestamp":1790306063041,"version":"4.1.0"},"reference-count":72,"publisher":"Elsevier BV","issue":"03","license":[{"start":{"date-parts":[[2023,7,10]],"date-time":"2023-07-10T00:00:00Z","timestamp":1688947200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"crossref","award":["81170472"],"award-info":[{"award-number":["81170472"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"crossref"}]},{"name":"Key Technology Research and Development Program of Tianjin China","award":["18ZXDBSY00140"],"award-info":[{"award-number":["18ZXDBSY00140"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Glob Med Genet"],"published-print":{"date-parts":[[2023,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a newly defined refractory adult-onset autoinflammatory syndrome caused by somatic mutations in the ubiquitin-like modifier-activating enzyme 1 (UBA1) gene in hematopoietic stem and progenitor cells, resulting in a shift in UBA1 isoform expression. Thus, patients develop a spectrum of systemic inflammatory manifestations and hematologic symptoms. To date, patients respond poorly to immune suppressive drugs, except high-dose glucocorticoids, and no treatment guidelines have been established. Given the high mortality rate, VEXAS syndrome needs to be taken seriously by physicians in all specialties. This article aims to describe the key features, pathogenesis, and clinical manifestations of VEXAS syndrome to better understand the targeted treatment and improve the prognosis of VEXAS syndrome.<\/jats:p>","DOI":"10.1055\/s-0043-1770958","type":"journal-article","created":{"date-parts":[[2023,7,26]],"date-time":"2023-07-26T17:30:26Z","timestamp":1690392626000},"page":"133-143","source":"Crossref","is-referenced-by-count":38,"title":["VEXAS Syndrome\u2014Review"],"prefix":"10.1016","volume":"10","author":[{"given":"Yue","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Hematology, General Hospital, Tianjin Medical University, Tianjin, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xifeng","family":"Dong","sequence":"additional","affiliation":[{"name":"Department of Hematology, General Hospital, Tianjin Medical University, Tianjin, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huaquan","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Hematology, General Hospital, Tianjin Medical University, Tianjin, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","published-online":{"date-parts":[[2023,7,10]]},"reference":[{"issue":"27","key":"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":"D B Beck","year":"2020","journal-title":"N Engl J Med"},{"issue":"08","key":"ref2","doi-asserted-by":"crossref","first-page":"435","DOI":"10.1038\/s41584-022-00778-4","article-title":"Disorders of ubiquitylation: unchained inflammation","volume":"18","author":"D B Beck","year":"2022","journal-title":"Nat Rev 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