Purpose: To report a case of non-paraneoplastic autoimmune retinopathy (npAIR) with phenotypical features of pericentral retinal degeneration (PRD) who responded to intravenous immunoglobulin therapy.

Methods: A case report.

Patient: A 27-year-old male presented with a recent subacute progressive nyctalopia and photopsia.

Results: Dilated fundoscopy demonstrated confluent yellow-white patches along the main temporal vascular arcades with sparing of the central island in the posterior pole. Color vision, fundus autofluorescence, fluorescein angiography, static visual field, and electroretinographic studies were inconclusive for retinal degeneration. Subsequent genetic testing for known mutations were negative. Work-up for paraneoplastic autoimmune retinopathy was negative. Anti-retinal antibodies were positive. The patient was diagnosed with non-paraneoplastic autoimmune retinopathy and was treated with intravenous immunoglobulin, which resulted in objective and subjective improvement on electroretinography, visual field, and optical coherence tomography of retina.

Conclusions: and importance: Non-paraneoplastic autoimmune retinopathy may present in a patient with the clinical phenotype of PRD. It is essential to rule out non-paraneoplastic autoimmune retinopathy in patients with subacute changes in the natural course of pericentral retinal degeneration, as treatment with intravenous immunoglobulin may be helpful.