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, venous thrombosis) and hematological impairment (giant cell anemia, thrombocytopenia, bone marrow and pre-erythrocyte vacuoles, bone marrow dysplasia), consequently contributing to significant morbidity and mortality. Current treatment management method is not well developed, and the main existing therapies are aimed at controlling inflammatory symptoms or targeting UBA1 mutations. Symptomatic supportive care includes control risk factors (such as infection and thrombosis), component transfusion, and use of hematopoietic drugs. This review aims to summarize new advances of the pathogenesis, clinical manifestations and treatment of this disease in the past two years.