Is it VEXAS or is it vasculitis?

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a recently defined clinical entity caused by somatic mutations in UBA1, a gene encoding the ubiquitin-activating enzyme 1, the major enzyme that initiates ubiquitylation. Dysfunction of the ubiquitin pathway results in a treatment-refractory autoinflammatory syndrome associated with haematologic abnormalities. Clinical features are heterogenous but small-, medium- and large-vessel vasculitis have been observed. This review will highlight vasculitic manifestations of VEXAS syndrome and provide recommendations on when to consider VEXAS syndrome among patients presenting with atypical, treatment-refractory, or recalcitrant vasculitic features.