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RADGONDE AMER, Khaled Safadi, Eyal Banin, Boris Rosin, Itay Chowers; Clinical, Electrophysiological and Immunological Features of Patients with Autoimmune Retinopathy. Invest. Ophthalmol. Vis. Sci. 2019;60(9):6675. doi: https://doi.org/.
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© ARVO (1962-2015); The Authors (2016-present)
Autoimmune retinopathy (AIR) is a rare, ill-defined entity that causes progressive vision loss due to retinal degeneration. It encompasses paraneoplastic and non-paraneoplastic entities. In this study, we aim to present our experience in diagnosing and managing patients with AIR.
A retrospective review of medical records of patients diagnosed with AIR between the years 2009-2017 was performed. Data on demographic, clinical, electrophysiological features in addition to results of serological testing for anti-retinal antibodies was collected.
Included were nine patients (6 males) with a mean age at presentation of 65 years. Five patients had previous cancer, of whom 3 had prostate cancer, one had colon cancer and one had non-small cell lung carcinoma. Previous malignancy was diagnosed at a mean of 11 years before diagnosis. Only two of them were actively treated for metastatic disease at the time of presentation. In 4 patients, extensive work-up did not reveal any underlying malignancy at presentation and all throughout the period of the follow-up. The median time interval between onset of ocular symptoms and diagnosis was 36 months. Mean follow-up time was 63 months. The most common symptoms were reduced vision and nyctalopia. Mean Snellen visual acuity at presentation was 0.5 and was 0.3 at last follow-up. The most common ocular signs included optic disc pallor, bone-spicule like pigmentation and retinal arteriolar attenuation in around half of the eyes. Optical conference tomography (OCT) was normal in 50% of the eyes. The most commonly encountered features on OCT were attenuated nerve fiber layer in 50% and attenuated outer nuclear layer and ellipsoid zone in 38% of the eyes. Electroretinography (ERG) demonstrated markedly reduced cone and rod amplitudes in all patients. Treatment included immunosuppression and plasmapheresis. After treatment, the ERG amplitudes stabilized in the majority and worsened in patients who stopped the treatment.
AIR is a diagnostic and therapeutic challenge to ophthalmologists. Diagnosis is based on a combination of clinically progressive disease with electrophysiological compromise and presence of circulating anti-retinal autoantibodies. Long-term immunosuppression is the mainstay of treatment, however, it is a condition associated with poor prognosis.
This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.
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