Tehran University of Medical Sciences Case Reports in Clinical Practice 2538-2683 2 3 2018 02 17 71 74 EN Asal Azami Rheumatologist, Department of Internal Medicine, Imam Reza Hospital, Mashhad University of Medical Sciences, Mashhad, Iran. Mohammad Hassan Jokar Department of Rheumatology, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran. 2017 08 22 2017 12 19 Systemic lupus erythematosus is an autoimmune disease. Neuromyelitis optica (Devic’s disease) is an inflammatory disorder belonging to the central nervous system. The typical characterizations include severe, immune-mediated demyelination, and axonal damage which mostly involve optic nerves and spinal cord, also the brain and brainstem. Anti-aquaporin- 4 antibody has been recently described as a highly specific marker for neuromyelitis optica. Neuromyelitis optica occasionally is linked with systemic autoimmune disorders, including systemic lupus erythematosus. Here, we describe a 26-year-old young woman with systemic lupus erythematosus who had bilateral optic neuritis with no evidence of myelitis or other core clinical criteria. However, aquaporin 4 antibody with high titer was detected. The patient received high-dose prednisolone, cyclophsphamid, and rituximab; but this treatment caused no change in her visual acuity. In patients with systemic lupus erythematosus who complicated by optic neuritis (with or without myelitis), the association of neuromyelitis optica should be considered. https://crcp.tums.ac.ir/index.php/crcp/article/view/130 https://crcp.tums.ac.ir/index.php/crcp/article/download/130/82