Autoimmune Retinopathy and Antiretinal Antibodies

Grewal, Dilraj S.; Fishman, Gerald A.; Jampol, Lee M.

doi:10.1097/01.iae.0000450880.26367.4e

Cited by 21 publications

(24 citation statements)

References 104 publications

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“…Ocular signs often precede the onset of systemic symptoms. [9][10][11][12][13][14] In the present case, the autofluorescence and the fluorescein patterns are consistent with the diagnosis of BDUMP. There was no obvious serous retinal detachment, but the scans available for review were performed through the fovea, and not at the level of the lesions.…”

supporting

confidence: 84%

Diagnostic and Therapeutic Challenges

Neamtu¹,

Eller²,

Cohen³

2016

Retina

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Case ReportThis is a case of a 58-year-old Asian woman who returned for ophthalmic evaluation of pigmentary retinal changes and routine Plaquenil screening. She initially presented at age 57, without any visual complaints. She was being treated with Plaquenil 200 mg daily for a total of 5 years for systemic lupus erythematosus. On initial presentation, her vision in both eyes was 20/20 and anterior segment examination was unremarkable. Dilated fundus examination revealed a bilateral pigmentary change that encompassed both nerves and extended into the macula, with asymmetry, in which the left eye ( Figure 1, C and D) showed more macular involvement than did the right eye ( Figure 1, A and B). At that time, she was only observed. It was deemed safe for her to continue Plaquenil, given that at the time, her lifetime dosage was 389 g over 5 years, and daily dosage calculated based on ideal body weight was 4 mgÁkg −1 Áday −1 .At follow-up evaluation, a year later at age 58, she had no visual complaints. Her Plaquenil lifetime dosage had increased to 469 g. Visual acuity was 20/20 in both eyes, and the anterior segment examination remained unremarkable. The repeat dilated fundus examination exhibited progression of retinopathy in both eyes ( Figure 2). The extent of the lesions is best demonstrated using fundus autofluorescence (Figure 2, B and D). Spectral domain optical coherence tomography (Spectralis) images (Figure 3) revealed discrete irregularities at the level of the retinal pigmented epithelium, but without associated loss of photoreceptor segment lines. Fluorescein angiography (Figure 4) was consistent with a speckled and punctate pattern of hyperfluorescence in the area of the lesions. Interestingly, indocyanine green angiography ( Figure 5) also highlighted the affected retina but with a pattern of hypofluorescence as contrasted to fluorescein angiography. In addition, indocyanine green angiography also showed punctate hyperfluorescent lesions outside those that were clinically apparent, indicating more widespread involvement.Laboratory testing was obtained to rule out infectious and inflammatory conditions, including quantiFERON gold, FTA-ABS, and Lyme serology, which were negative. A recent PPD read negative as well.This patient is presented for diagnosis, prognosis, and management, and specifically whether to continue Plaquenil therapy.

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supporting

confidence: 84%

Diagnostic and Therapeutic Challenges

Neamtu¹,

Eller²,

Cohen³

2016

Retina

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“…Autoimmune retinopathies (AIR) are rare but devastating autoimmune diseases in humans, characterized by the sudden onset of severe visual deficits (or complete blindness), extinguished retinal electrical activity, relatively normal fundus appearance, and presence of serum retinal autoantibodies 9‐11 . AIRs can develop as a form of paraneoplastic syndrome (ie, CAR: cancer‐associated retinopathy; MAR: melanoma‐associated retinopathy), or in the absence of cancer (nonparaneoplastic autoimmune retinopathies: npAIR) 12‐15 .…”

Section: Introductionmentioning

confidence: 99%

Presumed cancer‐associated retinopathy (CAR) mimicking Sudden Acquired Retinal Degeneration Syndrome (SARDS) in canines

Grozdanić

Lazic

Kecova

et al. 2020

Veterinary Ophthalmology

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Objective To describe functional and structural features of presumed cancer‐associated retinopathy (CAR) mimicking sudden acquired retinal degeneration syndrome (SARDS) in dogs and describe treatment outcomes. Animals Subjects were 17 dogs from 8 eight US states and Canada diagnosed with SARDS or immune‐mediated retinitis (IMR) by 12 ophthalmologists. Nine eyes from seven deceased patients were used for microarray (MA), histology, or immunohistochemical (IHC) analysis. Procedures Dogs underwent complete ophthalmic examination, including retinal photography, optical coherence tomography (OCT), chromatic pupil light reflex testing (cPLR), and electroretinography (ERG), in addition to complete systemic examination. Histology, microarray, and IHC analysis were performed in CAR retinas to evaluate histological and molecular changes in retinal tissue. Results None of the patients evaluated satisfied previously established criteria for diagnosis of SARDS (flat ERG+ no red – good blue PLR), and all were diagnosed with IMR. All patients were diagnosed with a cancer: meningioma (24%), sarcoma (18%), pituitary tumor (12%), and squamous cell carcinoma (12%), other (34%). Median survival time was 6 months from diagnosis (range 1‐36 months). Most frequent systemic abnormalities were as follows: proteinuria (78%); elevated liver enzymes (47%); and metabolic changes (PU/PD, polyphagia – 24%). Immunosuppressive therapy resulted in the reversal of blindness in 44% of treated patients, with 61% of all treated patients recovering and/or maintaining vision. Median time for preservation of vision was 5 months (range 1‐35 months). Conclusions Observed changes are highly suggestive of immune‐mediated damage in IMR‐CAR eyes. A relatively high percentage of patients with CAR responded positively to immunosuppressive therapy.

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“…These tests are mainly available in research facilities. Lack of an accepted gold standard for antiretinal antibodies detection alone makes their use in the diagnosis challenging 9 10. Postmortem examination of eyes with CAR have shown nearly complete loss of photoreceptor outer and inner segments and the outer nuclear layer in the retina 6…”

Section: Discussionmentioning

confidence: 99%

Thyroid malignancy presenting with visual loss: an unusual case of paraneoplastic retinopathy

Hughes¹,

Moran

Flitcroft

et al. 2016

BMJ Case Reports

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Paraneoplastic retinopathy is a rare cause of painless vision loss, associated with an underlying (and often occult) systemic malignancy. Ocular examination findings are subtle, and the diagnosis is often made on the basis of electrophysiology findings. This report describes the case of a 48-year-old Caucasian man with paraneoplastic retinopathy presenting as visual disturbance, central scotomata and abnormal electrophysiology. He was subsequently diagnosed with papillary thyroid malignancy.

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Autoimmune Retinopathy and Antiretinal Antibodies

Cited by 21 publications

References 104 publications

Diagnostic and Therapeutic Challenges

Diagnostic and Therapeutic Challenges

Presumed cancer‐associated retinopathy (CAR) mimicking Sudden Acquired Retinal Degeneration Syndrome (SARDS) in canines

Thyroid malignancy presenting with visual loss: an unusual case of paraneoplastic retinopathy

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