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To report the clinical presentation of acute visual loss in six patients who were ultimately diagnosed with systemic lupus erythematosus (SLE).Retrospective case series.All patients had a positive antinuclear antibody and elevated anti–double stranded DNA titers. Five of six patients demonstrated gadolinium enhancement of the optic nerve and/or chiasm on magnetic resonance imaging (MRI). Most patients showed initial improvement after treatment with high-dose systemic corticosteroids, but five experienced recrudescences during steroid taper, requiring further treatment with immunosuppressive or cytotoxic medications.Visual loss owing to optic neuropathy or chiasmopathy may be the presenting sign of SLE or the event that leads to this diagnosis. Gadolinium-enhanced MRI is useful for identifying anterior visual pathway lesions in these patients. Corticosteroids are effective in the treatment of this condition; however, relapses requiring further treatment are common.