Purpose: Autoimmune retinopathy (AIR) is a rare disorder that causes retinal degeneration via an immunologic mechanism. There is no established efficient treatment for AIR, which is often refractory to immunosuppression therapy. Here, we report a case of AIR diagnosed at an early-onset stage, in which early corticosteroid pulse therapy was effective.
Case: A 62-year-old male presented at our hospital with the subjective symptoms of bilateral decreased visual acuity (VA) and night blindness, which has started 3 days ago. His corrected VA was reduced to 20/32 OD and 20/25 OS. A visual field test showed concentric constriction of visual fields in both eyes. Optical coherence tomography (OCT) showed loss of the ellipsoid zone. Electroretinography examination revealed marked attenuation during photopic and scotopic conditions. Systemic examination showed no neoplasm, and serous anti-recoverin antibody was positive. Therefore, we diagnosed the patient with non-paraneoplastic AIR (npAIR) and treated him with intravenous methylprednisolone pulse therapy followed by oral administration of a 60-mg daily dose of prednisolone, which was gradually tapered. At 1-week post treatment, his VA, visual field, and OCT results were markedly improved, and there was no recurrence. One year after treatment, no neoplasm was detected.
Conclusions: A patient with npAIR improved with corticosteroid pulse therapy, possibly because of the short period between the appearance of subjective symptoms and hospital visit, resulting in early treatment initiation in the early stage of the disease. Our findings indicate that early corticosteroid pulse therapy may be effective for AIR.
Nippon Ganka Gakkai Zasshi (J Jpn Ophthalmol Soc) 122: 934-941, 2018.