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Autoimmune retinopathy and optic neuropathy associated with enolase-positive renal oncocytoma
Journal article   Open access   Peer reviewed

Autoimmune retinopathy and optic neuropathy associated with enolase-positive renal oncocytoma

Justine L Cheng, Johanna D Beebe, Kenneth G Nepple, Yousef Zakharia, Robert F Mullins, Miles J Flamme-Wiese, Matthew J Thurtell and Ian C Han
American journal of ophthalmology case reports, Vol.12, pp.55-60
12/2018
DOI: 10.1016/j.ajoc.2018.09.005
PMID: 30229140
url
https://doi.org/10.1016/j.ajoc.2018.09.005View
Published (Version of record) Open Access

Abstract

To report a case of autoimmune retinopathy and optic neuropathy associated with an enolase-positive renal oncocytoma. A 41-year-old man presented with subacute, painless, bilateral vision loss. On initial examination, visual acuity measured 20/125 OD and 20/1250 OS, and telangiectatic vessels were noted on the optic nerves and in the maculae. Goldmann perimetry showed bilateral, cecocentral scotomas, and electroretinography demonstrated reduced photopic and scotopic signals, concerning for autoimmune retinopathy. Serum testing showed multiple positive anti-optic nerve and anti-retinal antibodies, including to alpha-enolase. Extensive systemic workup was negative except for a large, exophytic, right renal mass. Biopsy was consistent with a benign oncocytoma, and immunohistochemical staining showed diffusely positive alpha-enolase staining. The patient was treated with a five-day course of intravenous methylprednisolone and plasmapheresis with minimal improvement. Surgical excision of the oncocytoma was performed. At 9-months post-operatively, visual acuity had improved to 20/40 OU, with corresponding improvement on visual field and electroretinography testing. To our knowledge, this is the first report of autoimmune retinopathy and optic neuropathy associated with a renal oncocytoma. The case highlights the importance of a thorough systemic workup in cases of suspected autoimmune retinopathy and reminds clinicians that even tumors considered benign can have distal effects on other organs.
Optic neuropathy Oncocytoma Autoimmune retinopathy

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