The ACR has published a formal international consensus guidance on VEXAS as a resource for clinicians seeking to understand the disease and its management. 

VEXAS is a rare genetic disorder; the acronym stands for Vacuoles E1 enzyme X-linked autoinflammatory somatic syndrome (VEXAS) and is associated with somatic mutations in the UBA1 gene. VEXAS presents with a combination of inflammatory and hematologic manifestations, leading to increased morbidity and mortality.