A "Leopard Man" Aspect on 18 F-FDG PET/CT Revealing a VEXAS Syndrome.
case_report · Level V
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- Record sourced from PubMed, PMID 36252945.
- Also identified by DOI 10.1097/RLU.0000000000004443.
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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.
Medical subject headings
- Polychondritis, Relapsing
- Giant Cell Arteritis