VEXAS syndrome
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
VEXAS syndrome. This is an autoinflammatory disorder. Fully referenced neurology checklist.
Pathology
- This is an autoinflammatory disorder
- It is caused by mutations in the ubiquitin-like modifier-activating enzyme 1 (UBA1) gene
- The gene is on the X chromosome
Epidemiology
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Systemic features
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Dermatological features
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Haematological features
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Respiratory features
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Rheumatological features
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Ophthalmological features
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Gastrointestinal features
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Cardiac features
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Neurological features
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Investigations
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Drug treatments
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Less effective drug treatments
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Non-drug treatments
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Acronym
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References
- Zhang Y, Dong X, Wang H. VEXAS syndrome - review. Glob Med Genet 2023; 10:133-143.
- Singh A, Chaudhary R. VEXAS syndrome: a newly identified X-Linked hematoinflammatory disorder - A comprehensive overview of its genetic, molecular, inflammatory, and clinical landscape. J Autoimmun 2025; 154:103425.
- Grayson PC, Patel BA, Young NS. VEXAS syndrome. Blood 2021; 137:3591-3594.
