Autoinflammatory & rare systemic diseases
Vasculitis associated with VEXAS syndrome
Authors' conclusion
VEXAS syndrome manifests as a variable vessel vasculitis in a quarter of patients, with cutaneous small and medium vessel involvement being particularly common.
Study context
- Population
- Patients with VEXAS syndrome verified by positive UBA1 mutation; all male, median age of onset 66.9 yearsall male
- Participants
- 89 patients
- Outcome
- Prevalence of confirmed vasculitis (histopathology showing vascular inflammation or non-invasive angiography findings)
Methodological detailschart review
- Chart review
Main finding
As reported in the abstract
Vasculitis · median follow-up 3.8 (IQR 2.2-5.5) years
Patients with evidence of vasculitis
21
23.6%
Observational data: associations, not effects.
Further findings
Vasculitis subtypes
- Small vessel vasculitis
- 19.1%
- Cutaneous medium vessel vasculitis
- 2.2%
- Large vessel vasculitis
- 2.2%
- No patient had more than one vessel size involved
Histopathology in small vessel vasculitis
- Leukocytoclastic peritubular capillaritis on renal biopsy
- 1
- Consistent with cutaneous leukocytoclastic vasculitis in the majority
Cranial symptoms (headache, vision changes or jaw pain)
- Cranial symptoms
- 18.0%
- Large vessel involvement with carotid thickening on non-invasive angiography, without cranial symptoms
- 2
- Positive temporal artery biopsy among these
- 1
Original publication
Vasculitis associated with VEXAS syndrome. Rheumatology (Oxford). Published 11 October 2024. doi:10.
Composed by AI from the published abstract and checked against it. How Briefs are made →