[VEXAS Syndrome].

VEXAS SYNDROME. VEXAS (Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic) syndrome is a recently described autoinflammatory syndrome, mostly affecting men above 50 years, caused by somatic mutation in the X-linked UBA1 gene. Patients present a broad spectrum of inflammatory manifestations (fever, neutrophilic dermatosis, chondritis, pulmonary infiltrates, ocular inflammation, venous thrombosis) with hematological involvement (macrocytic anemia, thrombocytopenia, vacuoles in myeloid and erythroid precursor cells, dysplastic bone marrow) which are responsible for significant morbidity and mortality. The therapeutic management is currently poorly codified, and based on two main approaches: controlling inflammatory symptoms by using corticosteroids, JAK inhibitors or tocilizumab, or targeting the UBA1-mutated hematopoietic population using azacitidine or allogeneic hematopoietic stem cell transplantation.