In a prospective study of 550 patients with systemic lupus erythematosus (SLE), 41 were found to have retinopathy. Lupus retinopathy in the majority of cases consisted of a microangiopathy, with an excellent prognosis for vision. Five patients developed other complications that resulted in loss of visual acuity. Lupus retinopathy was associated with active SLE in 88% of patients and with lupus cerebritis in 73% of cases. Retinopathy in SLE was a marker of poor prognosis for survival.