The eye in systemic vasculitis

This review summarizes the spectrum of ocular manifestations in systemic vasculitis, emphasizing recent advances in clinical recognition, multimodal imaging, biomarkers, and targeted treatment.

Ocular involvement may precede systemic manifestations and affect the anterior segment, retina, choroid, optic nerve, or orbit. In giant cell arteritis, vascular ultrasound is a preferred first-line imaging modality, while orbital and retinal imaging may provide complementary information on ischemic injury and treatment response. Ultra-widefield fluorescein angiography improves the detection of peripheral retinal nonperfusion in Takayasu arteritis. In antineutrophil cytoplasmic antibody-associated vasculitis, particularly granulomatosis with polyangiitis, ocular or orbital inflammation may be the initial or isolated manifestation, and rituximab-based therapy has been associated with favorable outcomes in orbital inflammatory disease. In Behçet uveitis, ocular-specific diagnostic criteria, imaging biomarkers, and treat-to-target biologic strategies are emerging. However, many of these approaches require validation in larger prospective studies.

Prompt recognition and multidisciplinary management are essential to prevent irreversible visual loss. New imaging methods and targeted therapies have broadened diagnostic and therapeutic options. Despite these advances, standardized ocular outcome measures and validation of emerging imaging and molecular biomarkers remain important priorities.