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

  • Inflammatory Disorders
  • Published:
Graefe's Archive for Clinical and Experimental Ophthalmology Aims and scope Submit manuscript

Abstract

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.

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Data availability

The corresponding author has full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis as well as the decision to submit for publication.

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional (HMO-Hadassah Medical Organization IRB approved the study) and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

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Authors and Affiliations

  1. Department of Ophthalmology, Hadassah Medical Organization and Faculty of Medicine, Hebrew University of Jerusalem, POB 12000, 91120, Jerusalem, Israel

    Khaled Safadi, Itay Chowers, Eyal Banin, Boris Rosin, Liran Tiosano & Radgonde Amer

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  1. Khaled Safadi
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  2. Itay Chowers
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  3. Eyal Banin
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  4. Boris Rosin
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Contributions

KS was involved in the design and conduct of the study, collection and analyzing of the data, and writing the manuscript. RA was involved in the management of the clinical cases, the design and conduct of the study, and writing the manuscript. IC, EB, BR, and LT were involved in analyzing the ERG tests and reviewing the manuscript.

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Correspondence to Radgonde Amer.

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The authors declare no competing interests.

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Safadi, K., Chowers, I., Banin, E. et al. Autoimmune retinopathy: clinical, electrophysiological, and immunological features in nine patients with long-term follow-up. Graefes Arch Clin Exp Ophthalmol 260, 975–991 (2022). https://doi.org/10.1007/s00417-021-05409-4

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  • DOI: https://doi.org/10.1007/s00417-021-05409-4

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