Abstract
VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) is a rare autoinflammatory disorder that manifests in older men and is caused by somatic mutations in the UBA1 gene, which encodes ubiquitin-like modifier-activating enzyme E1.1 Clinically, VEXAS syndrome is a multisystem disease characterized by constitutional symptoms, cutaneous and pulmonary disease, thromboembolic events, and myelodysplastic syndrome.2,3
