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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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CME Disclosure Statement: Unless noted, all individuals in control of content reported no relevant financial relationships. If applicable, all relevant financial relationships have been mitigated.
Corresponding Author: Emilia Maggio, MD, IRCCS Sacro Cuore Don Calabria Hospital, Via Don Sempreboni 5, Negrar 37024 Verona, Italy (firstname.lastname@example.org).
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.
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