Non-Paraneoplastic Autoimmune Retinopathy (npAIR)

A patient presented to the neuro-ophthalmology clinic for progressively decreasing vision in both eyes. He first noticed flashes and a blind spot in both eyes 3 months ago. Best-corrected visual acuity was 20/80 in the right eye and 20/50 in the left eye with normal intraocular pressures. Anterior segment exam was unremarkable and posterior exam revealed attenuated vasculature, pigmentary mottling in the periphery, and trace optic nerve pallor. Optical coherence tomography showed outer retinal and photoreceptor loss sparing the fovea while autofluorescence showed diffuse hyperautofluoresence in the macula. Ganglion cell layer showed diffuse macular thinning sparing the fovea and Humphrey visual field testing showed central ring scotoma. Electroretinogram showed decreased rod and cone function. Anti-retinal antibody panel revealed positive carbonic anhydrase, enolase, arrestin, and pkm2 antibodies. The patient underwent systemic work-up for malignancy which was negative and was subsequently diagnosed with non-paraneoplastic autoimmune retinopathy. He was treated with local steroid injection and oral methotrexate, with stabilization of his disease.

Presentation Date: 11/03/2022

Issue Date: 12/16/2022