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T. A. Larson, C. C. Gottlieb, W. M. Zein, R. B. Nussenblatt, H. N. Sen; Autoimmune Retinopathy: Prognosis and Treatment. Invest. Ophthalmol. Vis. Sci. 2010;51(13):6375.
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The diagnosis of autoimmune retinopathy (AIR) is difficult to make. This study was undertaken to describe the natural course of autoimmune retinopathy and evaluate response to immunomodulatory treatment in a cohort of patients.
Retrospective chart review of patients diagnosed with autoimmune retinopathy (AIR) at the National Eye Institute was performed. Diagnosis was based on history, exam findings, ERG and or visual field, and the presence of anti-retinal antibodies.
Seventeen patients were identified with a diagnosis of AIR. The mean follow up was 31 months. Four had paraneoplastic AIR; 2 had melanoma associated (MAR) and 2 had cancer associated retinopathy (CAR). The majority of cases occurred in women (15/17) with a mean age of 50 (32 - 69). Mean visual acuity (VA) at presentation was 20/50 and 20/40 and at last follow up was 20/63 and 20/60. Most common symptoms were subacute vision loss (71%) followed by photopsias (41%) and nyctalopia (24%). The most common fundus findings included retinal pigmentary changes (53%), CME (24%), and vascular attenuation (24%). Visual field changes were common (94%). All had ERG changes that showed variable levels of rod and/or cone dysfunction. Eight of the 12 non-paraneoplastic AIR patients received immunomodulatory treatment (IMT) consisting of periocular steroids, prednisone, cyclosporine, azathioprine, and rituximab infusions. Approximately 50% showed slight improvement in VA with treatment (with resolution of CME), 37% remained stable whereas one patient (12.5%) had a decline in VA despite treatment with a combination of cyclosporine, azathioprine, and adalimumab.
Autoimmune retinopathy is a rare disease that occurs predominantly in middle age women. Treatment with IMT helps stabilize vision in most patients. In patients with a suspected diagnosis of AIR in which a systemic workup is negative, IMT should be considered to stabilize vision loss.
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