Kristin Horwood ACNP-BC, AOCNP, MSN VEXAS Syndrome Overview VEXAS syndrome is a unique adult-onset disease with several varying clinical signs and symptoms. It is important for hematology/oncology advanced practice providers (APPs) to be aware of VEXAS syndrome as it is associated with several hematological disorders. V (vacuoles), E (E1 enzyme), X (X-linked), A (autoinflammatory), S (somatic mutation in ubiquitin-like modifier-activating enzyme 1 [UBA1] gene)1 syndrome was only recently described in 2020 as a new category of hemato-inflammatory diseases. This occurs from myeloid-driven inflammation arising from somatic mutations of the stem cell; in this case, mutations of the UBA1 gene.2 The syndrome is characterized by severe autoinflammation, which commonly presents with vasculitis, skin rash, polychondritis, arthritis, pulmonary inflammation, and constitutional symptoms. In a case series of 9 men with VEXAS syndrome, 88% had refractory constitutional symptoms that began 4 years prior to diagnosis.3 The diagnosis of VEXAS syndrome includes evidence of