Aim: To report a rare case of visual recovery and vascular reperfusion in a unilateral vasoocclusive retinopathy and retinal vasculitis presenting as the initial and only manifestation of systemic lupus erythematosus(SLE) despite elevated serological markers.
Materials & Methods: Retrospective case report
Results: A 33-year-old lady with no systemic complaints, presented with blurred vision in left eye(OS). She was diagnosed to have unilateral vasoocclusive retinopathy with retinal vasculitis and macular edema(OS). Fluorescein angiography and OCTA confirmed areas of multiple arteriolar occlusions(OS). She was diagnosed to have SLE on the basis of strongly positive antinuclear antibody, nRNP/Sm(3+), SS-A(2+) and Rib.Pprotein(2+), leucopenia(3400cells/mm3), increased ESR(68mm/hr) and low complement-3 levels. There were no other clinical signs of SLE. Antiphospholipid antibodies were negative. Treatment included pulse doses of intravenous methyl prednisolone followed by oral steroids, pulse doses of intravenous cyclophosphamide, posterior subtenon steroid injection and sectoral laser photocoagulation. Serial multimodal imaging showed vascular reperfusion and good visual outcome.
Conclusion: This case report highlights the excellent visual outcome and vascular reperfusion documented with multimodal imaging in a rare case of unilateral vasoocclusive retinopathy and retinal vasculitis occurring as an isolated finding in SLE despite elevated serum markers. These findings suggest that the vaso-occlusive disease is reversible if the diagnosis is made promptly and intensive therapy is initiated.