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Orbital inflammation in VEXAS syndrome
Clare Quigley1, James Pietris2, Terence Ang3
1Department of Ophthalmology, Royal Adelaide Hospital, Adelaide, South Australia, Australia quigleyclare@gmail.com.
The British Journal of Ophthalmology
|January 14, 2026
Summary
Vacuoles, E1-ligase, X-linked Auto-inflammatory, Somatic (VEXAS) syndrome frequently causes orbital inflammation and eyelid swelling in affected males. While vision is often preserved, severe vision loss can occur, necessitating prompt ophthalmic evaluation.
Area of Science:
- Ophthalmology
- Rheumatology
- Genetics
Background:
- Vacuoles, E1-ligase, X-linked Auto-inflammatory, Somatic (VEXAS) syndrome is a recently identified multisystem inflammatory disorder.
- Ocular manifestations of VEXAS syndrome are not well-characterized, necessitating further investigation.
Purpose of the Study:
- To describe the ocular and orbital features in patients diagnosed with VEXAS syndrome.
- To highlight the spectrum of eye involvement in this patient population.
Main Methods:
- A case series was compiled from ophthalmologists associated with prominent ophthalmic surgical societies.
- Data from 14 male patients with VEXAS syndrome and documented eye features were analyzed.
Main Results:
- All 14 patients presented with periorbital edema; eyelid swelling and eye pain were also common symptoms.
- Orbital inflammation, including dacryoadenitis and orbital myositis, was observed in a majority of cases.
- While most patients maintained normal visual acuity, 14% experienced unilateral severe vision loss due to complications like orbital compartment syndrome.
Conclusions:
- Orbital inflammation is a consistent finding in VEXAS syndrome with ocular involvement.
- Ocular symptoms in VEXAS patients warrant urgent ophthalmological assessment due to the risk of severe vision loss.
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