UBA1 and VEXAS syndrome: VEXAS syndrome ('Vacuoles', 'E1 enzyme', 'X-linked', 'Autoinflammatory' and 'Somatic') is a rare autoinflammatory disorder caused by somatic mutations in the <i>UBA1</i> (ubiquitin-like modifier-activating enzyme 1) gene whose treatment and prognosis remain poorly understood.