VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome was first described in late 2020. It occurs as the result of somatic mutations in the ubiquitin-activating enzyme UBA1. Since its discovery, there have been numerous articles describing typical features of VEXAS, including alveolitis, polychondritis of the ear and nose, and thromboembolic conditions. We report a case of cardiac involvement that manifested as heart failure with nonischemic cardiomyopathy in a patient with VEXAS syndrome.
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Xu et al. (2024) studied this question.
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