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Clinical Heterogeneity of the VEXAS Syndrome

Dr. Matthew Koster, a Clinical Rheumatologist from Mayo Clinic in Rochester, MN, reviews his article appearing in the October 2021 issue of Mayo Clinic Proceedings where he describes nine cases of a newly discovered autoinflammatory disease syndrome called VEXAS. The name is comprised of the clinical features seen in patients including vacuoles, E1 enzyme, X-linked, autoinflammatory conditions, and somatic origin. It is caused by a somatic mutation in the UBA1 gene with a notably diverse clinical presentation. Macrocytic anemia, male sex, and symptoms refractory to traditional steroid-sparing agents should prompt clinicians to consider VEXAS. Available at: https://mayocl.in/3mAbqMR