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Autoimmune retinopathy: A review and summary

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Abstract

Three main forms of autoimmune retinopathy (AIR) have been identified over the last 15 years: cancer-associated retinopathy (CAR), melanoma-associated retinopathy (MAR), and nonneoplastic autoimmune retinopathy (npAIR). In this chapter, the term AIR will be used to encompass all three disorders where there is commonality to their features. Complicating the issue is that AIR can be a secondary complication of other conditions such as retinitis pigmentosa, ocular trauma, birdshot retinopathy, acute zonal occult outer retinopathy (AZOOR), or multiple evanescent white dot syndrome (MEWDS). The many forms of AIR tend to have common clinical features despite the fact that there has been no uniform set of anti-retinal antibodies circulating in these patients. Patients tend to have a wide variance of anti-retinal antibody activity often with three to six different antibodies found on immunoblots. Patients typically present with a sudden onset of photopsia, rapid visual loss, and abnormal electroretinograms (ERGs). Most patients have a panretinal degeneration without pigment deposits.

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Acknowledgments

Monique Leys, MD referred the patient with MAR, and the authors gratefully acknowledge Lynn Gordon, MD, PhD, Natalia Aptsiauri, MD, PhD, Shirley He, MD, Ying Lu, MD, Elena Filippova, MD, and Robert Nussenblatt, MD for discussions, help with Western blots, and ELISA testing.

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Authors and Affiliations

  1. Kellogg Eye Center, University of Michigan, Ann Arbor, MI, 48105, USA

    John R. Heckenlively

  2. Shiley Eye Center, University of California, San Diego, La Jolla, CA, 92093, USA

    Henry A. Ferreyra

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  1. John R. Heckenlively
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  2. Henry A. Ferreyra
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Correspondence to John R. Heckenlively.

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Heckenlively, J.R., Ferreyra, H.A. Autoimmune retinopathy: A review and summary. Semin Immunopathol 30, 127–134 (2008). https://doi.org/10.1007/s00281-008-0114-7

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  • DOI: https://doi.org/10.1007/s00281-008-0114-7

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