Aortitis in VEXAS Syndrome

A 74-year-old man was referred for fever of unknown origin lasting four weeks.The patient had no cranial symptoms, including auricular chondritis.Large-vessel giant cell arteritis was suspected because the aorta showed uptake on fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) (Picture 1, 2, arrows).The fever abated with corticosteroid therapy, but recurred after dosage reduction.Macrocytic anemia and vacuoles in the bone marrow erythroblasts led to the suspicion of vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syn-