[New Advances in the Study of VEXAS Syndrome --Review].

Abstract excerpt

Vacuoles, E1 enzyme, X-linked, autoinflammatory, and somatic syndrome (VEXAS) is a recently discovered adult-onset autoinflammatory syndrome characterized by methionine somatic mutations affecting the activation of ubiquitin system in the X-linked gene UBA1 . Patients present with a wide range of inflammatory manifestations (fever, neutrophil dermatosis, chondritis, pulmonary infiltrates, ocular inflammation,...

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