The Sleeping Wolf - Widefield OCT Angiography Revelations of Insidious Systemic Lupus Erythematous-Associated Retinal Vasculitis

Purpose: To describe a case of minimally symptomatic, insidious systemic-lupus-erythematous (SLE) retinal vasculitis revealed with widefield multimodal imaging. Methods: Report of a case. Results: A 56-year-old woman presented with a vague history of visual blur in her left eye for 3 weeks. While her central acuity was relatively preserved, fundus examination was notable for scattered cotton-wool spots in a Purtscher-like retinopathy presentation. Widefield angiographic imaging revealed profound peripheral non-perfusion with some focal arteriolar vasculitis. Systemic work-up confirmed the diagnosis of SLE and high-dose intravenous steroid therapy was initiated. Immune modulatory therapy with mycophenolate mofetil and rituximab infusions were subsequently added to achieve control. Conclusions: Retinal vasculitis is a rare manifestation of SLE which may present in the absence of, or prior to central nervous system involvement. Visual prognosis is extremely guarded, necessitating aggressive immunosuppression and often pan-retinal photocoagulation. Widefield optical coherence tomography angiography may have a role in screening otherwise asymptomatic SLE patients for retinopathy as well as utility in monitoring treatment response.