Research
Ocular Involvement in VEXAS Syndrome: Insights from AIDA Network VEXAS Registry
An international cohort analysis explores patterns, prognostic and clinical implications of orbital/ocular inflammatory manifestations.
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Based on 59 patients enrolled in the international AIDA Network registry dedicated to VEXAS, this new study highlights the intersection of VEXAS syndrome with ocular inflammation. In particular, the protean spectrum of orbital and ocular manifestations was analysed, revealing a significant prevalence (45.8%) of such involvement, coinciding with disease onset in almost the totality of patients.
Notable manifestations include periorbital edema, episcleritis, scleritis, uveitis, conjunctivitis, and blepharitis, with certain patients experiencing multiple afflictions. Importantly, a significant association between ocular involvement and concomitant signs compatible with relapsing polychondritis emerges. Notably, most of the patients who died during follow-up also suffered from ocular/orbital involvement, with consequent prognostic implications.
These findings suggest a possible increased mortality risk among patients with ocular features, emphasizing the need for heightened clinical vigilance in patients with ophthalmologic involvement. Therefore, beyond limitations in sample size, the study underscores the pivotal role of ophthalmologists in diagnosing and managing VEXAS syndrome, advocating for comprehensive evaluation and vigilant monitoring in affected individuals.