Events

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is a rare late-onset autoinflammatory disorder caused by somatic mutations in the UBA1 gene. Although most reported cases of VEXAS manifest as severe multi-organ inflammation, we will present a case with atypical clinical manifestations. We will then discuss recent mechanistic insight into this mysterious disorder, diagnostic approaches, and advances in therapeutic management.