{"id":820,"date":"2026-03-30T07:06:35","date_gmt":"2026-03-30T07:06:35","guid":{"rendered":"https:\/\/batnajms.net\/?p=820"},"modified":"2026-03-30T07:06:57","modified_gmt":"2026-03-30T07:06:57","slug":"ababsa-mouaki","status":"publish","type":"post","link":"https:\/\/batnajms.net\/index.php\/2026\/03\/30\/ababsa-mouaki\/","title":{"rendered":"VEXAS syndrome\u00a0: literature review"},"content":{"rendered":"\n<h3 class=\"wp-block-heading\" id=\"block-c2f138d4-0208-4b99-af09-93c83eb26d35\"><strong><a href=\"https:\/\/batnajms.net\/wp-content\/uploads\/Archives\/2026\/04\/BJMS_Ababsa-Mouaki.pdf\" target=\"_blank\" rel=\"noreferrer noopener\"><strong>Fulltext\u00a0<\/strong><\/a>(PDF)<\/strong><\/h3>\n\n\n\n<p><strong>DOI<\/strong>: 10.48087\/BJMS.2026.130410<\/p>\n\n\n\n<p><strong>Authors:<\/strong>\u00a0Abdelhak ABABSA MOUAKI, Ouassila ABBASI<\/p>\n\n\n\n<p><strong>Affiliations:<\/strong>\u00a0Service de m\u00e9decine interne EHS Athmana Mahmoud, Batna \u2013 Alg\u00e9rie<\/p>\n\n\n\n<p><strong>Abstract<\/strong><\/p>\n\n\n\n<p>VEXAS is an acronym standing for vacuoles, enzyme E1, X-linked, autoinflammatory, and somatic. It designates an autoinflammatory syndrome in adults, first described in 2020, linked to acquired somatic mutations in the <em>UBA1<\/em> gene (<em>ubiquitin-activating enzyme E1<\/em>), located on the X chromosome and restricted to the hematopoietic compartment. The syndrome primarily affects men over 50 years of age, combining systemic inflammatory manifestations with suggestive hematological abnormalities. VEXAS syndrome should be considered in male patients presenting with incomplete or complete features of relapsing polychondritis, polyarteritis nodosa, giant cell arteritis, or Sweet&rsquo;s syndrome \u2014 particularly when refractory to treatment and associated with hematological involvement such as cytopenias, myelodysplastic syndrome, or thromboembolic disease. Diagnosis relies on the identification of pathogenic variants of <em>UBA1<\/em> \u2014 primarily p.Met41 \u2014 in blood or bone marrow. Management remains poorly defined and is based on two complementary approaches: anti-inflammatory treatment (corticosteroids, tocilizumab, JAK inhibitors) and\/or targeting of the mutated hematopoietic clone (azacitidine, allogeneic hematopoietic stem cell transplantation), combined with appropriate supportive care.<\/p>\n\n\n\n<p><strong>_____________________________________________________________<\/strong><strong><\/strong><\/p>\n\n\n\n<p><strong>Keywords<\/strong><strong>: <\/strong>VEXAS syndrome, UBA1, Myelodysplastic syndrome, Autoinflammatory diseases.<\/p>\n","protected":false},"excerpt":{"rendered":"<p>Fulltext\u00a0(PDF) DOI: 10.48087\/BJMS.2026.130410 Authors:\u00a0Abdelhak ABABSA MOUAKI, Ouassila ABBASI Affiliations:\u00a0Service de m\u00e9decine interne EHS Athmana Mahmoud, Batna \u2013 Alg\u00e9rie Abstract VEXAS is an acronym standing for vacuoles, enzyme E1, X-linked, autoinflammatory, and somatic. It designates an autoinflammatory syndrome in adults, first described in 2020, linked to acquired somatic mutations in the UBA1 gene (ubiquitin-activating enzyme E1), &hellip; <a href=\"https:\/\/batnajms.net\/index.php\/2026\/03\/30\/ababsa-mouaki\/\" class=\"more-link\">Continuer la lecture de <span class=\"screen-reader-text\">VEXAS syndrome\u00a0: literature review<\/span><\/a><\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[1],"tags":[],"class_list":["post-820","post","type-post","status-publish","format-standard","hentry","category-uncategorized"],"jetpack_featured_media_url":"","_links":{"self":[{"href":"https:\/\/batnajms.net\/index.php\/wp-json\/wp\/v2\/posts\/820","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/batnajms.net\/index.php\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/batnajms.net\/index.php\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/batnajms.net\/index.php\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/batnajms.net\/index.php\/wp-json\/wp\/v2\/comments?post=820"}],"version-history":[{"count":1,"href":"https:\/\/batnajms.net\/index.php\/wp-json\/wp\/v2\/posts\/820\/revisions"}],"predecessor-version":[{"id":821,"href":"https:\/\/batnajms.net\/index.php\/wp-json\/wp\/v2\/posts\/820\/revisions\/821"}],"wp:attachment":[{"href":"https:\/\/batnajms.net\/index.php\/wp-json\/wp\/v2\/media?parent=820"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/batnajms.net\/index.php\/wp-json\/wp\/v2\/categories?post=820"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/batnajms.net\/index.php\/wp-json\/wp\/v2\/tags?post=820"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}