VEXAS syndrome

Evidence-based neurology checklist on vexas syndrome: 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 Systemic features Dermatological features Haematological…

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

Systemic features

Dermatological features

Haematological features

Respiratory features

Rheumatological features

Ophthalmological features

Gastrointestinal features

Cardiac features

Neurological features

Investigations

Drug treatments

Less effective drug treatments

Non-drug treatments

Acronym

References

  1. Zhang Y, Dong X, Wang H. VEXAS syndrome - review. Glob Med Genet 2023; 10:133-143.
  2. 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.
  3. Grayson PC, Patel BA, Young NS. VEXAS syndrome. Blood 2021; 137:3591-3594.

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