{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T09:35:02Z","timestamp":1792143302670,"version":"4.4.0"},"reference-count":52,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,8,11]],"date-time":"2025-08-11T00:00:00Z","timestamp":1754870400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2025,8,11]],"date-time":"2025-08-11T00:00:00Z","timestamp":1754870400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>\n                      Vacuoles E1 enzyme X\u2010linked autoinflammatory somatic syndrome (VEXAS) is a recently identified rare genetic disorder associated with somatic mutations in the\n                      <jats:italic>UBA1<\/jats:italic>\n                      gene. VEXAS presents with a combination of inflammatory and hematologic manifestations, leading to increased morbidity and mortality.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Given the variability in disease presentation and the limited number of studies to date, no clinical documents currently exist to provide guidance to health care providers about the management of VEXAS. To address this gap, we formed an international multidisciplinary panel of VEXAS experts.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Through formalized meetings and a voting process, the group developed consensus clinical guidance considerations for the management of VEXAS. These considerations offer practical advice on several key topics: (1) clinical features of VEXAS, (2)\n                      <jats:italic>UBA1<\/jats:italic>\n                      screening methods, (3) the diagnosis of myelodysplastic syndromes (MDSs) in patients with VEXAS, and (4) prognosis and management. The aim is to provide expert guidance on which patients to test, how to test for VEXAS, how to approach MDS in the context of VEXAS, and considerations for management.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>This work marks the first formal international consensus guidance for VEXAS and is intended to be used as a resource for clinicians seeking to understand the disease and its management.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/art.43287","type":"journal-article","created":{"date-parts":[[2025,8,11]],"date-time":"2025-08-11T12:00:16Z","timestamp":1754913616000},"page":"509-522","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":37,"title":["American College of Rheumatology Guidance Statement for Diagnosis and Management of\n                    <scp>VEXAS<\/scp>\n                    Developed by the International\n                    <scp>VEXAS<\/scp>\n                    Working Group Expert Panel"],"prefix":"10.1002","volume":"78","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2849-3049","authenticated-orcid":false,"given":"Arsene","family":"Mekinian","sequence":"first","affiliation":[{"name":"Service de m\u00e9decine interne, H\u00f4pital Saint Antoine, Assistance Publique Hopitaux de Paris, Sorbonne Universit\u00e9, Imagine Institute, CEREMAIAA reference center  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sophie","family":"Georgin\u2010Lavialle","sequence":"additional","affiliation":[{"name":"Internal Medicine Department Hopital Tenon, Assistance Publique Hopitaux de Paris, Sorbonne University, CEREMAIAA reference center  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcela A.","family":"Ferrada","sequence":"additional","affiliation":[{"name":"Division of Rheumatology and Immunology, Department of Medicine Baltimore, University of Maryland  USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sinisa","family":"Savic","sequence":"additional","affiliation":[{"name":"Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds  Leeds United Kingdom"},{"name":"National Institute for Health and Care Research (NIHR)\u2010Leeds Biomedical Research Centre  Leeds United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2895-6755","authenticated-orcid":false,"given":"Matthew J.","family":"Koster","sequence":"additional","affiliation":[{"name":"Division of Rheumatology Mayo Clinic  Rochester MN USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Olivier","family":"Kosmider","sequence":"additional","affiliation":[{"name":"Universit\u00e9 de Paris Cit\u00e9, Institut Cochin, CNRS UMR8104, INSERM U1016  Paris France"},{"name":"Hematology Laboratory Cochin Hopital, 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