Autoimmune retinopathy

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Disease Overview

Autoimmune retinopathy represents a spectrum of rare autoimmune diseases that primarily affect retinal photoreceptor function and lead to progressive vision loss. Included in this spectrum are cancer-associated retinopathy (CAR), melanoma-associated retinopathy (MAR) and presumed non-paraneoplastic autoimmune retinopathy (npAIR). Autoimmune retinopathy typically presents in the fifth and sixth decades with rapidly progressive, bilateral, painless visual deterioration. Examination of the fundus (the back inner part of the eye) is usually normal at presentation. [7266][7267] The underlying reason for the autoimmune attack on retinal cells remains unknown. Treatment includes systemic immunosuppression with steroid and steroid-sparing agents, intravenous immunoglobulin (IVIG), and plasmapheresis (plasma exchange).[7266] 



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National Organization for Rare Disorders