J Korean Ophthalmol Soc.  2015 Jul;56(7):1134-1140. 10.3341/jkos.2015.56.7.1134.

A Case of Nonparaneoplastic Autoimmune Retinopathy

• Eo DR
• Yun JH
• Kim SJ

• KMID: 2148817
• DOI: http://doi.org/10.3341/jkos.2015.56.7.1134

Abstract

PURPOSE
To report a case of nonparaneoplastic autoimmune retinopathy diagnosed using serum anti-retinal autoantibodies.
CASE SUMMARY
A 60-year-old female complained of progressive visual loss in both eyes over 3 months. Her best corrected visual acuity was hand motion in the right eye, 0.9 (decimal) in the left eye, and no definite abnormal findings were identified on fundus examinations. Automated visual field test revealed severely depressed visual fields in both eyes. Standard full-field electroretinogram (ERG) revealed nearly extinguished scotopic b-waves and spectral-domain optical coherence tomography (SD-OCT) showed subtle obscuration and interruption of the inner segment/outer segment (IS/OS) junction of the photoreceptors. Using Western blotting with human retinal proteins and the patient's serum, we diagnosed nonparaneoplastic autoimmune retinopathy and performed posterior subtenon steroid injection in the right eye, systemic corticosteroids, and oral mycophenolate mofetil. Full-field ERG after treatment showed slightly increased amplitude but there was no subjective visual improvement. OCT after treatment did not reveal significant changes in the photoreceptor layer.
CONCLUSIONS
This is the first reported case of nonparaneoplastic autoimmune retinopathy in Korea diagnosed using Western blotting with anti-retinal autoantibodies. Autoimmune retinopathy should be considered in patients with visual field and ERG impairment without definite fundus abnormalities.

Keyword

Anti-retinal autoantibody; Nonparaneoplastic autoimmune retinopathy; Western blot

MeSH Terms

Adrenal Cortex Hormones
Autoantibodies
Blotting, Western
Female
Hand
Humans
Korea
Middle Aged
Retinaldehyde
Tomography, Optical Coherence
Visual Acuity
Visual Field Tests
Visual Fields
Adrenal Cortex Hormones
Autoantibodies
Retinaldehyde

Figure

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  Figure 1. (A, B) Normal fundus photographs. (C) Fundus autofluorescence reveals a ring of outer hyperautofluorescence (between round dots and arrows) in the right eye. (D) Fundus autofluorescence showed no remarkable findings in the left eye.

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  Figure 2. Automated visual field test revealed severely de-pressed visual fields in the (A) right eye and (B) left eye (more in the right eye than in the left eye).

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  Figure 3. Standard full field ERG reveals nearly extinguished scotopic a and b waves, negative waves of combined responses, and severely de-creased amplitude of photopic response in both eyes. ERG = electroretinogram.

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  Figure 4. (A) Multifocal electroretinogram shows decreased P1 amplitude and increased implicit time which is more prominent in the right eye. (B) Normal multifocal electroretinogram in the left eye. T = temporal; I = inferior; N =nasal; S = superior.

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  Figure 5. Spectral-domain coherence tomography (SD-OCT) shows subtle obscuration and interruption of the inner seg-ment/outer segment (IS/OS) junction of the photoreceptors in the right eye (A) and in the left eye (B) (arrows).

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  Figure 6. Western blot analysis-Bands at 50 kDa were constantly seen in all lanes and reflect immunoglobulin G (IgG) heavy chains present in the soluble protein extracts from human tissue. Arrows indicate proteins that react with serum from pa-tient, not with serum from control. Band A near 46 kDa and band B near 39 kDa.

Reference

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