Autoinflammatory & rare systemic diseases
Clinical features in VEXAS syndrome: a systematic review
Authors' conclusion
This systematic review provides the most comprehensive characterization of VEXAS syndrome to date, establishing robust prevalence estimates across all organ systems and identifying rare manifestations with important clinical implications.
Study context
- Population
- Genetically confirmed VEXAS syndrome cases published since its discovery in 202033 case reports · 21 case series
- Participants
- 720 participants
- Outcome
- Prevalence of clinical manifestations across organ systems
Methodological detailsPRISMA guidelines, five databases, Wilson score intervals, 32 countries
- PRISMA guidelines
- Five databases
- Wilson score intervals
- 32 countries
Pooled result
As reported in the abstract
Prevalence of manifestations
- Cutaneous involvement (predominant), 95% CI 78.8-84.5%
- 81.8%
- Constitutional symptoms, 95% CI 66.0-72.7%
- 69.4%
- Respiratory disease, 95% CI 57.6-64.7%
- 61.3%
- Joint involvement, 95% CI 43.5-51.2%
- 47.3%
- Ocular disease, 95% CI 40.5-48.2%
- 44.3%
- VTE, 95% CI 38.3-45.4%
- 41.8%
- Myelodysplastic syndrome, 95% CI 32.3-39.4%
- 35.8%
Further pooled results
Rare but clinically significant features
- Cardiac involvement
- 7.6%
- Renal disease
- 7.0%
- CNS manifestations
- 7.8%
Original publication
Clinical features in VEXAS syndrome: a systematic review. Rheumatology (Oxford). Published 16 June 2025. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →