{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T03:57:28Z","timestamp":1792468648180,"version":"4.5.0"},"reference-count":31,"publisher":"Elsevier BV","issue":"5","license":[{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-017"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-037"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-012"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-029"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"stm-asf","delay-in-days":0,"URL":"https:\/\/doi.org\/10.15223\/policy-004"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Autoimmunity Reviews"],"published-print":{"date-parts":[[2026,5]]},"DOI":"10.1016\/j.autrev.2026.104030","type":"journal-article","created":{"date-parts":[[2026,3,21]],"date-time":"2026-03-21T15:58:16Z","timestamp":1774108696000},"page":"104030","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":3,"title":["VEXAS syndrome in adults: A narrative review of genomic pathogenesis, multisystem phenotypes, diagnostic algorithms, and emerging therapeutic strategies"],"prefix":"10.1016","volume":"25","author":[{"given":"Antonio","family":"Vitale","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlos","family":"Cifuentes-Gonz\u00e1lez","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Martina","family":"Gentile","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Cataldi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kajal","family":"Agrawal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rupesh","family":"Agrawal","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alessandro","family":"Conforti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Luca","family":"Cantarini","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"26","key":"10.1016\/j.autrev.2026.104030_bb0005","first-page":"3591","article-title":"VEXAS syndrome","volume":"137","author":"Grayson","year":"2021","journal-title":"Blood"},{"issue":"2","key":"10.1016\/j.autrev.2026.104030_bb0010","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1080\/1744666X.2023.2157262","article-title":"An update on VEXAS syndrome","volume":"19","author":"Al-Hakim","year":"2022","journal-title":"Expert Rev Clin Immunol"},{"key":"10.1016\/j.autrev.2026.104030_bb0015","first-page":"1","article-title":"VEXAS syndrome","author":"Nakajima","year":"2024","journal-title":"Int J Hematol"},{"key":"10.1016\/j.autrev.2026.104030_bb0020","doi-asserted-by":"crossref","DOI":"10.1136\/rmdopen-2023-003332","article-title":"VEXAS syndrome: a diagnostic puzzle","volume":"9","author":"Ruffer","year":"2023","journal-title":"RMD Open"},{"issue":"3","key":"10.1016\/j.autrev.2026.104030_bb0025","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1055\/s-0043-1770958","article-title":"VEXAS syndrome-review","volume":"10","author":"Zhang","year":"2023","journal-title":"Glob Med Genet"},{"key":"10.1016\/j.autrev.2026.104030_bb0030","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1097\/BOR.0000000000001068","article-title":"VEXAS syndrome: an adult-onset autoinflammatory disorder with underlying somatic mutation","volume":"37","author":"K\u00f6tter","year":"2024","journal-title":"Curr Opin Rheumatol"},{"issue":"8","key":"10.1016\/j.autrev.2026.104030_bb0035","doi-asserted-by":"crossref","first-page":"2665","DOI":"10.1093\/rheumatology\/keac712","article-title":"Diagnostic and therapeutic algorithms for monogenic autoinflammatory diseases presenting with recurrent fevers among adults","volume":"62","author":"Delplanque","year":"2023","journal-title":"Rheumatology"},{"key":"10.1016\/j.autrev.2026.104030_bb0040","article-title":"VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) for the dermatologist","author":"Sterling","year":"2022","journal-title":"J Am Acad Dermatol"},{"issue":"27","key":"10.1016\/j.autrev.2026.104030_bb0045","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"},{"issue":"4","key":"10.1016\/j.autrev.2026.104030_bb0050","doi-asserted-by":"crossref","first-page":"318","DOI":"10.1001\/jama.2022.24836","article-title":"Estimated prevalence and clinical manifestations of UBA1 variants associated with VEXAS syndrome in a clinical population","volume":"329","author":"Beck","year":"2023","journal-title":"Jama"},{"key":"10.1016\/j.autrev.2026.104030_bb0055","doi-asserted-by":"crossref","DOI":"10.1016\/j.semarthrit.2024.152430","article-title":"Orbital\/ocular inflammatory involvement in VEXAS syndrome: data from the international AIDA network VEXAS registry","volume":"66","author":"Vitale","year":"2024","journal-title":"Semin Arthritis Rheum"},{"key":"10.1016\/j.autrev.2026.104030_bb0060","article-title":"Development and implementation of the AIDA international registry for patients with VEXAS syndrome","volume":"9","author":"Vitale","year":"2022","journal-title":"Front Med"},{"issue":"4","key":"10.1016\/j.autrev.2026.104030_bb0065","doi-asserted-by":"crossref","DOI":"10.1016\/j.jbspin.2024.105700","article-title":"VEXAS syndrome: an update","volume":"91","author":"Khitri","year":"2024","journal-title":"Joint Bone Spine"},{"issue":"7","key":"10.1016\/j.autrev.2026.104030_bb0070","doi-asserted-by":"crossref","first-page":"1219","DOI":"10.1007\/s00296-023-05513-0","article-title":"Clinical characteristics, disease trajectories and management of vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome: a systematic review","volume":"44","author":"Kouranloo","year":"2024","journal-title":"Rheumatol Int"},{"issue":"21","key":"10.1016\/j.autrev.2026.104030_bb0075","doi-asserted-by":"crossref","first-page":"2190","DOI":"10.1182\/blood.2023022329","article-title":"Venous and arterial thrombosis in patients with VEXAS syndrome","volume":"143","author":"Kusne","year":"2024","journal-title":"Blood"},{"issue":"1","key":"10.1016\/j.autrev.2026.104030_bb0080","doi-asserted-by":"crossref","first-page":"910","DOI":"10.1038\/s41467-024-44811-4","article-title":"VEXAS syndrome is characterized by inflammasome activation and monocyte dysregulation","volume":"15","author":"Kosmider","year":"2024","journal-title":"Nat Commun"},{"issue":"14","key":"10.1016\/j.autrev.2026.104030_bb0085","doi-asserted-by":"crossref","first-page":"1193","DOI":"10.1182\/blood.2022018581","article-title":"Single-cell genomics in acquired bone marrow failure syndromes","volume":"142","author":"Wu","year":"2023","journal-title":"Blood"},{"issue":"1","key":"10.1016\/j.autrev.2026.104030_bb0090","article-title":"Clinical manifestations in vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome: a narrative review","volume":"16","author":"Padureanu","year":"2024","journal-title":"Cureus"},{"issue":"3","key":"10.1016\/j.autrev.2026.104030_bb0095","doi-asserted-by":"crossref","first-page":"170","DOI":"10.5582\/irdr.2023.01020","article-title":"VEXAS syndrome: current clinical, diagnostic and treatment approaches","volume":"12","author":"Kobak","year":"2023","journal-title":"Intract Rare Dis Res"},{"issue":"3","key":"10.1016\/j.autrev.2026.104030_bb0100","doi-asserted-by":"crossref","first-page":"564","DOI":"10.1111\/bjd.20805","article-title":"Further characterization of clinical and laboratory features in VEXAS syndrome: large-scale analysis of a multicentre case series of 116 French patients","volume":"186","author":"Georgin-Lavialle","year":"2022","journal-title":"Br J Dermatol"},{"issue":"3","key":"10.1016\/j.autrev.2026.104030_bb0105","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1136\/ard-2023-224819","article-title":"Serious infections in patients with VEXAS syndrome: data from the French VEXAS registry","volume":"83","author":"de Valence","year":"2024","journal-title":"Ann Rheum Dis"},{"key":"10.1016\/j.autrev.2026.104030_bb0110","article-title":"Efficacy and safety profile of biotechnological agents and Janus kinase inhibitors in VEXAS syndrome: data from the international AIDA network VEXAS registry","volume":"16","author":"Vitale","year":"2025","journal-title":"Front Pharmacol"},{"key":"10.1016\/j.autrev.2026.104030_bb0115","doi-asserted-by":"crossref","DOI":"10.3389\/fphar.2025.1539756","article-title":"Efficacy and safety of conventional disease-modifying antirheumatic drugs in VEXAS syndrome: real-world data from the international AIDA network","volume":"16","author":"Vitale","year":"2025","journal-title":"Front Pharmacol"},{"issue":"1","key":"10.1016\/j.autrev.2026.104030_bb0120","doi-asserted-by":"crossref","first-page":"47","DOI":"10.1016\/j.reumae.2023.12.004","article-title":"VEXAS syndrome: clinical manifestations, diagnosis, and treatment","volume":"20","author":"Loeza-Uribe","year":"2024","journal-title":"Reumatol Cl\u00edn (Engl Ed)"},{"issue":"2","key":"10.1016\/j.autrev.2026.104030_bb0125","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1002\/ajh.27156","article-title":"VEXAS syndrome: clinical, hematologic features and a practical approach to diagnosis and management","volume":"99","author":"Koster","year":"2024","journal-title":"Am J Hematol"},{"issue":"11","key":"10.1016\/j.autrev.2026.104030_bb0130","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"},{"issue":"4","key":"10.1016\/j.autrev.2026.104030_bb0135","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1053\/j.seminhematol.2021.09.001","article-title":"Toward a pathophysiology inspired treatment of VEXAS syndrome","volume":"58","author":"Heiblig","year":"2021","journal-title":"Semin Hematol"},{"issue":"10","key":"10.1016\/j.autrev.2026.104030_bb0140","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"},{"issue":"11","key":"10.1016\/j.autrev.2026.104030_bb0145","doi-asserted-by":"crossref","first-page":"e402","DOI":"10.1093\/rheumatology\/keab392","article-title":"VEXAS syndrome in a woman","volume":"60","author":"Barba","year":"2021","journal-title":"Rheumatology"},{"issue":"22","key":"10.1016\/j.autrev.2026.104030_bb0150","doi-asserted-by":"crossref","first-page":"6970","DOI":"10.3390\/jcm13226970","article-title":"VEXAS syndrome: a comprehensive review of current therapeutic strategies and emerging treatments","volume":"13","author":"Alqatari","year":"2024","journal-title":"J Clin Med"},{"issue":"1","key":"10.1016\/j.autrev.2026.104030_bb0155","article-title":"Navigating therapeutic challenges in VEXAS syndrome: exploring IL-6 and JAK inhibitors at the forefront","volume":"30","author":"Li","year":"2024","journal-title":"Mol Med (Cambridge, Mass)"}],"container-title":["Autoimmunity Reviews"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1568997226000443?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1568997226000443?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2026,4,25]],"date-time":"2026-04-25T15:19:27Z","timestamp":1777130367000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1568997226000443"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5]]},"references-count":31,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2026,5]]}},"alternative-id":["S1568997226000443"],"URL":"https:\/\/doi.org\/10.1016\/j.autrev.2026.104030","relation":{},"ISSN":["1568-9972"],"issn-type":[{"value":"1568-9972","type":"print"}],"subject":[],"published":{"date-parts":[[2026,5]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"VEXAS syndrome in adults: A narrative review of genomic pathogenesis, multisystem phenotypes, diagnostic algorithms, and emerging therapeutic strategies","name":"articletitle","label":"Article Title"},{"value":"Autoimmunity Reviews","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.autrev.2026.104030","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2026 Published by Elsevier B.V.","name":"copyright","label":"Copyright"}],"article-number":"104030"}}