Non-paraneoplastic autoimmune retinopathy: The first case report in korea

Eun Young Choi, Min Kim, Grazyna Adamus, Hyoung Jun Koh, Sung Chul Lee

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

Autoimmune retinopathy (AIR) is an immune-mediated retinopathy, resulting from an immunologic process caused by the aberrant recognition of retinal antigens as autoantigens. The diagnosis of AIR involves the detection of antiretinal antibodies with concurrent clinical and electrophysiological evidence of retinopathy. A 40-year-old patient presented with progressive loss of bilateral vision over several months. A fundus examination was unremarkable. Spectral domain optical coherence tomography revealed a blurred photoreceptor ellipsoid zone at the subfoveal region in both eyes with more prominent disruption in the left eye. Full-field electroretinography (ERG) showed relatively normal rod and cone responses in the right eye, and decreased photopic b-waves with minimal attenuation of a-waves in the left eye. Multifocal ERG demonstrated slightly reduced amplitude of the inner segment ring in the right eye and decreased amplitudes and delayed latencies of all modalities in the left eye. The patient was suspected to have AIR and it was supported by positive Western blots for 23-kDa protein, enolase (46-kDa), aldolase (40-kDa), 62- kDa and 78-kDa proteins and by immunohistochemical staining of human retinal bipolar and ganglion cells. Despite the immunosuppressive treatment, the destruction of the retinal photoreceptors progressed, and immunosuppressive interventions produced very little visual improvement. We report on what is, to the best of our knowledge, the very first case of serologically confirmed nonparaneoplastic AIR in Korea.

Original languageEnglish (US)
Pages (from-to)527-531
Number of pages5
JournalYonsei Medical Journal
Volume57
Issue number2
DOIs
StatePublished - Mar 1 2016

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Korea
Electroretinography
Vertebrate Photoreceptor Cells
Immunosuppressive Agents
Retinal Bipolar Cells
Fructose-Bisphosphate Aldolase
Retinal Ganglion Cells
Phosphopyruvate Hydratase
Autoantigens
Optical Coherence Tomography
Proteins
Western Blotting
Staining and Labeling
Antigens
Antibodies

Keywords

  • AIR
  • Antiretinal antibodies
  • Autoimmune retinopathy
  • Enolase
  • Nonparaneoplastic autoimmune retinopathy
  • Recoverin

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Non-paraneoplastic autoimmune retinopathy : The first case report in korea. / Choi, Eun Young; Kim, Min; Adamus, Grazyna; Koh, Hyoung Jun; Lee, Sung Chul.

In: Yonsei Medical Journal, Vol. 57, No. 2, 01.03.2016, p. 527-531.

Research output: Contribution to journalArticle

Choi, Eun Young ; Kim, Min ; Adamus, Grazyna ; Koh, Hyoung Jun ; Lee, Sung Chul. / Non-paraneoplastic autoimmune retinopathy : The first case report in korea. In: Yonsei Medical Journal. 2016 ; Vol. 57, No. 2. pp. 527-531.
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