AI Article Synopsis
- VEXAS syndrome is an adult-onset autoinflammatory disease caused by mutations in the UBA1 gene, first identified in 2020.
- Patients typically present symptoms of inflammatory syndromes like relapsing polychondritis or hematologic conditions like myelodysplastic syndrome.
- A case report describes a 70-year-old man with VEXAS syndrome showing a unique "leopard man" appearance on FDG PET/CT imaging, indicating abnormal bone marrow activity.
Article Abstract
VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome is an adult-onset autoinflammatory disease caused by somatic UBA1 mutations first described in 2020. Most of these patients met clinical criteria for an inflammatory syndrome (relapsing polychondritis, Sweet syndrome, polyarteritis nodosa, or giant-cell arteritis) or a hematologic condition (myelodysplastic syndrome or multiple myeloma) or both. We described here an FDG PET/CT "leopard man" appearance, with abnormal marrow recruitment the findings, in a 70-year-old man diagnosed with a VEXAS syndrome.
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Article Synopsis
- VEXAS syndrome is an adult-onset autoinflammatory disease caused by mutations in the UBA1 gene, first identified in 2020.
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- A case report describes a 70-year-old man with VEXAS syndrome showing a unique "leopard man" appearance on FDG PET/CT imaging, indicating abnormal bone marrow activity.
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