Bilateral central retinal artery occlusion as a presenting manifestation of systemic lupus erythematosus: a case-based review.

Central retinal artery occlusion (CRAO) is an ophthalmic emergency that typically results in permanent vision damage even despite vigorous treatment. In this article, we describe a case of acute vaso-occlusive retinopathy that presented as the primary manifestation of SLE in the absence of elevated levels of APLAs. After treatment with intravenous steroids, immunoglobulin, intrathecal injection of dexamethasone, plasma exchange, and intravenous cyclophosphamide, SLE was well controlled in the patient, but her vision was permanently lost in the left eye. We also go over a brief review of the currently available literature on retinal vaso-occlusive disease present in SLE. The pathology mechanism of CRAO is related to immune complex-mediated "vasculitis", which is typically associated with neuropsychiatric lupus. However, the literature review identified antiphospholipid antibody syndrome (APS) in only 6 of 19 patients, indicating that other mechanisms besides APS are associated with CRAO. Systemic immunosuppression and anticoagulants are required for the treatment of this severe vaso-occlusive retinopathy. Early recognition and aggressive intervention may prevent severe loss of vision.