Occlusive retinal vasculitis is a form of retinopathy in systemic immune disorders. Aggressive therapy may promote vascular reperfusion and visual rehabilitation, even in severe cases.
A man in his 20s presented with bilateral decreased visual acuity (VA) after hospitalization for lupus cerebritis. Initial VA was 20/800 OD and counting fingers at the face OS. Fundus fluorescein angiography showed global nonperfusion in each eye with arteriolar involvement (Figure, A). Results were positive for antinuclear antibodies and anti-Smith antibodies and negative for antiphospholipid syndrome (APS), lupus anticoagulant, and anti–Sjögren syndrome–related antigen A and B (anti-SSA/SSB) antibodies. He began therapy with pulse methylprednisolone sodium succinate, 1 g/d intravenously for 3 days, and then transitioned to oral prednisone, 20 mg, with twice daily mycophenolate mofetil, 1000 mg, and cyclophosphamide, 50 mg. Within a week, VA improved to 20/300 OD and 20/200 OS with angiographic reperfusion. One week later, he received dexamethasone intravitreal implants (0.7 mg) in each eye, followed by panretinal photocoagulation (PRP) and intravitreous bevacizumab (1.25 mg) in each eye for neovascularization. Cyclosporine, 3 mg/kg/d, with mycophenolate mofetil, 40 mg/kg/d, replaced cyclophosphamide after 7 months. Over 28 months, he received 8 anti–vascular endothelial growth factor (VEGF) injections for cystoid macular edema and vitrectomy for epiretinal membranes. His VA stabilized at 20/100 OD and 20/80 OS, coincident with revascularization noted on angiography (Figure, B).
Bomar JG, Branson SV, McClintic J, Kurup SK. Reperfusion of Retinal Vasculature With Improved Visual Acuity in Ischemic Occlusive Vasculitis. JAMA Ophthalmol. 2016;134(4):466-467. doi:10.1001/jamaophthalmol.2015.6164