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Bilateral Central and Peripheral Vision Loss in an Otherwise Asymptomatic Woman

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 65-year-old woman was referred for symptoms of painless and progressive bilateral loss of central and peripheral vision over the last 6 months, associated with floaters in the left eye. The patient reported difficulty in performing essential daily tasks, such as driving and walking without assistance. She denied photopsias. Her previous ocular and medical history were unremarkable. She denied any systemic symptoms as well as any family history of inherited ocular diseases.

On examination, best-corrected visual acuity was 20/32 OU. Fundus examination results showed attenuated arterial and venous retinal vessels and slight retinal pigmented epithelium changes at the posterior pole, consisting of atrophy and pigment migration in both eyes, associated with vitreous opacities in the left eye. Fluorescein angiography examination results showed a mild leakage of arterial and venous retinal vasculature (Figure). Spectral-domain optical coherence tomography exhibited disruption at the photoreceptor level and multifocal hyperreflective material depositions at the retinal pigmented epithelium level.

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Paraneoplastic autoimmune retinopathy

C. Investigate serum positivity for antiretinal antibodies and obtain an extensive workup to rule out a malignant neoplasm

Autoimmune retinopathy (AIR) is a rare form of immune-mediated retinal degeneration. It is characterized by progressive vision loss along with electroretinographic, visual field abnormalities, and the presence of circulating ARAs. Autoimmune retinopathy can be categorized as paraneoplastic (pAIR), which includes cancer-associated retinopathy and melanoma-associated retinopathy, or nonparaneoplastic in the absence of a malignant neoplasm.1,2

Diagnosis remains a challenge because of its variable presentation and fundus appearance, which may be initially normal or with minimal unrevealing alterations despite central vision loss and a severely decreased visual field.1,2 Moreover, in contrast with previous reported cases,3,4 the patient did not show any systemic symptoms that would have been helpful toward the diagnosis.

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Article Information

Corresponding Author: Emilia Maggio, MD, IRCCS Sacro Cuore Don Calabria Hospital, Via Don Sempreboni 5, Negrar 37024 Verona, Italy (emi_maggio@yahoo.it).

Published Online: May 13, 2021. doi:10.1001/jamaophthalmol.2020.5278

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
1.
Sen  HN , Grange  L , Akanda  M , Fox  A . Autoimmune retinopathy: current concepts and practices (an American Ophthalmological Society thesis). Trans Am Ophthalmol Soc. 2018 Mar 8;115:T8.
2.
Fox  AR , Gordon  LK , Heckenlively  JR ,  et al.  Consensus on the diagnosis and management of nonparaneoplastic autoimmune retinopathy using a modified Delphi approach.   Am J Ophthalmol. 2016;168:183-190. doi:10.1016/j.ajo.2016.05.013 PubMedGoogle ScholarCrossref
3.
Micieli  JA , Lam  K , Margolin  EA .  Vision loss with postmenopausal bleeding.   JAMA Ophthalmol. 2016;134(7):831-832. doi:10.1001/jamaophthalmol.2015.5005 PubMedGoogle ScholarCrossref
4.
Rayess  N , Patel  VR .  Bilateral severe decreased vision with normal examination findings.   JAMA Ophthalmol. 2018;136(10):1201-1202. doi:10.1001/jamaophthalmol.2018.0485 PubMedGoogle ScholarCrossref
5.
Wiley  LA , Binkley  EM , DeLuca  AP ,  et al.  Autoimmune retinopathy mimicking heritable retinal degeneration in a patient with common variable immune deficiency.   Retin Cases Brief Rep. 2019. doi:10.1097/ICB.0000000000000941 PubMedGoogle Scholar
6.
Hooks  JJ , Tso  MO , Detrick  B .  Retinopathies associated with antiretinal antibodies.   Clin Diagn Lab Immunol. 2001;8(5):853-858. doi:10.1128/CDLI.8.5.853-858.2001 PubMedGoogle ScholarCrossref
7.
Lee  JH , Cho  SB , Bang  D ,  et al.  Human anti-alpha-enolase antibody in sera from patients with Behçet’s disease and rheumatologic disorders.   Clin Exp Rheumatol. 2009;27(2)(suppl 53):S63-S66.PubMedGoogle Scholar
8.
Adamus  G , Ren  G , Weleber  RG .  Autoantibodies against retinal proteins in paraneoplastic and autoimmune retinopathy.   BMC Ophthalmol. 2004;4:5. doi:10.1186/1471-2415-4-5 PubMedGoogle ScholarCrossref
9.
Adamus  G .  Antirecoverin antibodies and autoimmune retinopathy.   Arch Ophthalmol. 2000;118(11):1577-1578. doi:10.1001/archopht.118.11.1577 PubMedGoogle ScholarCrossref
10.
Weleber  RG , Watzke  RC , Shults  WT ,  et al.  Clinical and electrophysiologic characterization of paraneoplastic and autoimmune retinopathies associated with antienolase antibodies.   Am J Ophthalmol. 2005;139(5):780-794. doi:10.1016/j.ajo.2004.12.104 PubMedGoogle ScholarCrossref
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