Autoinflammatory & rare systemic diseases
Orbital/ocular inflammatory involvement in VEXAS syndrome: Data from the international AIDA network VEXAS registry
Authors' conclusion
In conclusion, this study confirms that orbital/ocular inflammatory involvement is a common finding in VEXAS patients, especially when relapsing polychondritis is diagnosed.
Study context
- Population
- VEXAS syndrome patients in the international AIDA network VEXAS registry
- Participants
- 59 patients
- Outcome
- Inflammatory orbital/ocular involvement during clinical history
Methodological detailsinternational AIDA network VEXAS registry
- International AIDA network VEXAS registry
Main finding
As reported in the abstract
Inflammatory orbital/ocular involvement
- Orbital/ocular inflammatory involvement
- 27 (45.8 %)
- Periorbital edema
- 8 (13.6 %)
- Episcleritis
- 5 (8.5 %)
- Scleritis
- 5 (8.5 %)
- Uveitis
- 4 (6.8 %)
- Conjunctivitis
- 4 (6.8 %)
- Blepharitis
- 3 (5.1 %)
- Orbital myositis
- 2 (3.4 %)
Observational data: associations, not effects.
Further findings
Association between relapsing polychondritis and orbital/ocular involvement in VEXAS syndrome
| Relative Risk (95 % CI) | P value | ||
|---|---|---|---|
| Relapsing polychondritis | 2.37 (1.03–5.46) | P = 0.048 | |
| 0.20.5125Relative Risk (log scale) |
Most frequent orbital/ocular affections
- Periorbital edema
- 8 (13.6 %)
- Episcleritis
- 5 (8.5 %)
- Scleritis
- 5 (8.5 %)
- Uveitis
- 4 (6.8 %)
- Conjunctivitis
- 4 (6.8 %)
- Blepharitis
- 3 (5.1 %)
- Orbital myositis
- 2 (3.4 %)
Systemic immune-mediated disease diagnosis
- Systemic immune-mediated disease
- 15 (55.6 %)
- Relapsing polychondritis
- 12
Deaths in the whole cohort · median disease duration of 1.2 (IQR=5.35) years
- Deaths
- Six
- Deaths with orbital/ocular inflammatory involvement
- 5 (83.3 %)
Original publication
Orbital/ocular inflammatory involvement in VEXAS syndrome: Data from the international AIDA network VEXAS registry. Semin Arthritis Rheum. Published 18 March 2024. doi:10.
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