To report a case of severe vaso-occlusive retinopathy with significant decrease of bilateral visual acuity as the first manifestation associated with systemic lupus erythematosus (SLE).

A 23-year-old man was referred to our clinic with bilateral visual impairment of hand motion (HH). Fundus examination revealed severe retinal hemorrhage, cotton-wool patch, occlusive retinal vasculitis with vascular engorgement, and diffuse retinal edema in both eyes. Because of a malar rash on both cheeks, generalized edema was observed on initial examination with hypertension, azotemia, anemia, and thrombocytopenia, The patient was diagnosed with SLE, strongly positive to antinuclear antibody (ANA), and received an intravitreal injection of Bevacizumab (Avastin, Genentech Inc., San Francisco, CA, USA) in the left eye in addition to hemodialysis, transfusion, systemic corticosteroid and immunosuppressant treatment due to lupus nephritis. Eighteen months later, the retinal edema, cotton-wool patch and hemorrhage resolved, leaving epiretinal membrane without traction in his left eye and diffuse degeneration of the right eye. Final visual acuity was HM in the right eye and 20/100 in the left eye.

Vaso-occlusive retinopathy in SLE can result in permanent visual impairment. In a patient with a high possibility of SLE retinopathy, a periodic fundus examination and intensive management of systemic disease should be considered.