{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,9]],"date-time":"2026-06-09T14:30:25Z","timestamp":1781015425129,"version":"3.54.1"},"reference-count":24,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T00:00:00Z","timestamp":1773360000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Recurrent febrile episodes account for one of the most frequent symptoms observed in Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic (VEXAS) syndrome and a key target for therapeutic intervention. Therefore, this study aims at investigating the association between recurrent febrile episodes and specific clinical manifestations, mortality and response to treatment.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Data were obtained from the international AutoInflammatory Disease\u00a0Alliance (AIDA) Network registry and analyzed using a Bayesian statistical approach. Posterior probabilities [P(\u03b2)] were calculated to assess the likelihood that fever was associated with clinical, laboratory, genetic, and therapeutic features.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>In total, 87 VEXAS patients were enrolled, 65 (74.7%) of whom suffered from recurrent fever episodes. Fever episodes showed a significant association with patients\u2019 mortality [P(\u03b2): 99.41%], as well as with major inflammatory organ involvement, including cardiac [P(\u03b2): 99.99%], lung [P(\u03b2): 99.98%], and gastrointestinal [P(\u03b2): 97.5%] involvement. The occurrence of recurrent fever episodes was associated with a negligible probability of both complete response and treatment failure [P(\u03b2) &amp;lt;2.5%], instead favoring a partial response [P(\u03b2) &amp;gt;97.5%] to conventional disease modifying anti-rheumatic drugs, Janus Kinases inhibitors, and tocilizumab. For temperatures exceeding 40 \u00b0C, using anti-interleukin-1 agents was associated with a high probability of treatment failure [P(\u03b2): 99.3%].<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>febrile episodes are associated with more severe pattern of organ involvement and, accordingly, to death. Furthermore, febrile episodes could correlate with differential therapeutic responsiveness, thereby potentially serving as a valuable marker to guide the treatment strategies in VEXAS patients.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fimmu.2026.1753412","type":"journal-article","created":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T06:49:13Z","timestamp":1773384553000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":1,"title":["Recurrent fever and association with severe organ involvement, mortality and treatment outcomes in VEXAS syndrome: data from the AIDA Network"],"prefix":"10.3389","volume":"17","author":[{"given":"Valeria","family":"Caggiano","sequence":"first","affiliation":[{"name":"Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Beh\u00e7et\u2019s Disease Clinic, University of Siena","place":["Siena, Italy"]},{"name":"Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center]","place":["Siena, Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessica","family":"Sbalchiero","sequence":"additional","affiliation":[{"name":"Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Beh\u00e7et\u2019s Disease Clinic, University of Siena","place":["Siena, Italy"]},{"name":"Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center]","place":["Siena, Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Micol","family":"Frassi","sequence":"additional","affiliation":[{"name":"Rheumatology and Clinical Immunology, Spedali Civili and Department of Clinical and Experimental Sciences, University of 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Italy"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Hinojosa-Azaola","sequence":"additional","affiliation":[{"name":"Department of Immunology and Rheumatology, Instituto Nacional de Ciencias M\u00e9dicas y Nutrici\u00f3n Salvador Zubir\u00e1n","place":["Mexico City, Mexico"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eduardo","family":"Mart\u00edn-Nares","sequence":"additional","affiliation":[{"name":"Department of Immunology and Rheumatology, Instituto Nacional de Ciencias M\u00e9dicas y Nutrici\u00f3n Salvador Zubir\u00e1n","place":["Mexico City, Mexico"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Guillermo Arturo","family":"Guaracha-Basa\u00f1ez","sequence":"additional","affiliation":[{"name":"Department of Immunology and Rheumatology, Instituto Nacional de Ciencias M\u00e9dicas y Nutrici\u00f3n Salvador Zubir\u00e1n","place":["Mexico City, 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