Autoimmune retinopathy: clinical, electrophysiological, and immunological features in nine patients with long-term follow-up

Purpose: We aim to report on the clinical, imaging, immunological, and electrophysiological features of patients with autoimmune retinopathy (AIR) with long-term follow-up. Methods: Single-center, retrospective study of a consecutive group of AIR patients treated in a tertiary academic medical center. Results: Included were nine patients with a mean ± SD age at presentation of 65 ± 13 years and a median follow-up of 63 months (range 18–120). Five patients were known to have cancer. Median interval between onset of ocular symptoms and diagnosis of AIR was 36 months. Mean baseline and final LogMAR visual acuity were 0.72 ± 0.9 and 1.1 ± 1.2, respectively (p = 0.17). The most common funduscopic findings included optic atrophy and bone-spicule-like pigmentation. Thinning of the nerve fiber layer was the most frequent optical coherence tomographic abnormality. Electroretinographic (ERG) recordings demonstrated variably reduced cone- and rod-derived amplitudes in the majority of eyes at presentation. The most commonly detected anti-retinal antibody was anti-α-enolase. Treatment included immunomodulatory therapy and plasmapheresis. ERG tests showed stability in 64% of eyes throughout the treatment period. Conclusion: This study highlights the importance of maintaining a high index of suspicion of AIR, particularly in late middle-aged and elderly patients with “unexplained” visual loss, in light of the non-specific posterior segment signs and the inconsistency of the routinely used ancillary tests.