Prevalence of Antiretinal Antibodies in Acute Zonal Occult Outer Retinopathy: A Comprehensive Review of 25 Cases

Cynthia X. Qian, Angeline Wang, David L. DeMill, Thiran Jayasundera, Kari Branham, Maria Fernanda Abalem, Naheed Khan, John R. Heckenlively

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

Purpose To perform a comprehensive review and to investigate the presence and role of autoimmune antibodies in 25 cases of acute zonal occult outer retinopathy (AZOOR) identified using the classification originally proposed by J. Donald Gass. Design Observational case series. Methods SETTING: Institutional. STUDY POPULATION: Twenty-five patients were identified by characteristic symptoms (abrupt onset of photopsias, followed by large scotomata at or connected to the blind spot), ocular findings (paucity of pigmentary changes with no sign of vitreous inflammation and abnormal electroretinogram in at least 1 eye), and a negative family history for retinitis pigmentosa. OBSERVATION PROCEDURES: Patients underwent a full comprehensive ophthalmologic examination, fundus retinography, Goldmann kinetic visual field (GVF), and full-field electroretinogram (ffERG). Blood samples were also obtained to verify for the presence of antiretinal antibodies by Western blot analysis. Main OUTCOME MEASURES: Clinical presentation, best-corrected visual acuity (BCVA), fundus abnormalities, visual field defects, ffERG changes, and presence of antiretinal antibodies. Results Sixteen patients (64%) presented with photopsias, 56% (14/25) with night blindness, and 56% (14/25) with loss of peripheral vision. Sixty-four percent (16/25) of cases were bilateral. All patients demonstrated retinal vascular attenuation, optic nerve head pallor, and mottling of retinal pigment epithelium. The most common visual field changes included enlargement and expansion of the blind spot extending into large pericentral or other types of scotomata (64%). Both scotopic and photopic ffERG values were abnormal and affected to a similar degree in our patients. Nine patients (36%) had a greater than 20% asymmetry in ERG values between the 2 eyes. All patients had antiretinal antibodies on Western blot with an average of 6.6 bands. Conclusion Evidence suggests that AZOOR is a unique form of autoimmune retinopathy and retinal manifestation suggests possible antiretinal antibody leakage from the disc margin with spread of immune products under the retina, resulting in large scotomata that connect to the optic nerve head.

Original languageEnglish (US)
Pages (from-to)210-218
Number of pages9
JournalAmerican journal of ophthalmology
Volume176
DOIs
StatePublished - Apr 1 2017
Externally publishedYes

Fingerprint

Optic Disk
Antibodies
Scotoma
Visual Fields
Western Blotting
Night Blindness
Pallor
Retinal Vessels
Retinitis Pigmentosa
Retinal Pigment Epithelium
Acute zonal occult outer retinopathy
Visual Acuity
Retina
Observation
Inflammation

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Prevalence of Antiretinal Antibodies in Acute Zonal Occult Outer Retinopathy : A Comprehensive Review of 25 Cases. / Qian, Cynthia X.; Wang, Angeline; DeMill, David L.; Jayasundera, Thiran; Branham, Kari; Abalem, Maria Fernanda; Khan, Naheed; Heckenlively, John R.

In: American journal of ophthalmology, Vol. 176, 01.04.2017, p. 210-218.

Research output: Contribution to journalArticle

Qian, Cynthia X. ; Wang, Angeline ; DeMill, David L. ; Jayasundera, Thiran ; Branham, Kari ; Abalem, Maria Fernanda ; Khan, Naheed ; Heckenlively, John R. / Prevalence of Antiretinal Antibodies in Acute Zonal Occult Outer Retinopathy : A Comprehensive Review of 25 Cases. In: American journal of ophthalmology. 2017 ; Vol. 176. pp. 210-218.
@article{8c7e909af9154f12ae0eee0fc52d5a8c,
title = "Prevalence of Antiretinal Antibodies in Acute Zonal Occult Outer Retinopathy: A Comprehensive Review of 25 Cases",
abstract = "Purpose To perform a comprehensive review and to investigate the presence and role of autoimmune antibodies in 25 cases of acute zonal occult outer retinopathy (AZOOR) identified using the classification originally proposed by J. Donald Gass. Design Observational case series. Methods SETTING: Institutional. STUDY POPULATION: Twenty-five patients were identified by characteristic symptoms (abrupt onset of photopsias, followed by large scotomata at or connected to the blind spot), ocular findings (paucity of pigmentary changes with no sign of vitreous inflammation and abnormal electroretinogram in at least 1 eye), and a negative family history for retinitis pigmentosa. OBSERVATION PROCEDURES: Patients underwent a full comprehensive ophthalmologic examination, fundus retinography, Goldmann kinetic visual field (GVF), and full-field electroretinogram (ffERG). Blood samples were also obtained to verify for the presence of antiretinal antibodies by Western blot analysis. Main OUTCOME MEASURES: Clinical presentation, best-corrected visual acuity (BCVA), fundus abnormalities, visual field defects, ffERG changes, and presence of antiretinal antibodies. Results Sixteen patients (64{\%}) presented with photopsias, 56{\%} (14/25) with night blindness, and 56{\%} (14/25) with loss of peripheral vision. Sixty-four percent (16/25) of cases were bilateral. All patients demonstrated retinal vascular attenuation, optic nerve head pallor, and mottling of retinal pigment epithelium. The most common visual field changes included enlargement and expansion of the blind spot extending into large pericentral or other types of scotomata (64{\%}). Both scotopic and photopic ffERG values were abnormal and affected to a similar degree in our patients. Nine patients (36{\%}) had a greater than 20{\%} asymmetry in ERG values between the 2 eyes. All patients had antiretinal antibodies on Western blot with an average of 6.6 bands. Conclusion Evidence suggests that AZOOR is a unique form of autoimmune retinopathy and retinal manifestation suggests possible antiretinal antibody leakage from the disc margin with spread of immune products under the retina, resulting in large scotomata that connect to the optic nerve head.",
author = "Qian, {Cynthia X.} and Angeline Wang and DeMill, {David L.} and Thiran Jayasundera and Kari Branham and Abalem, {Maria Fernanda} and Naheed Khan and Heckenlively, {John R.}",
year = "2017",
month = "4",
day = "1",
doi = "10.1016/j.ajo.2016.12.001",
language = "English (US)",
volume = "176",
pages = "210--218",
journal = "American Journal of Ophthalmology",
issn = "0002-9394",
publisher = "Elsevier USA",

}

TY - JOUR

T1 - Prevalence of Antiretinal Antibodies in Acute Zonal Occult Outer Retinopathy

T2 - A Comprehensive Review of 25 Cases

AU - Qian, Cynthia X.

AU - Wang, Angeline

AU - DeMill, David L.

AU - Jayasundera, Thiran

AU - Branham, Kari

AU - Abalem, Maria Fernanda

AU - Khan, Naheed

AU - Heckenlively, John R.

PY - 2017/4/1

Y1 - 2017/4/1

N2 - Purpose To perform a comprehensive review and to investigate the presence and role of autoimmune antibodies in 25 cases of acute zonal occult outer retinopathy (AZOOR) identified using the classification originally proposed by J. Donald Gass. Design Observational case series. Methods SETTING: Institutional. STUDY POPULATION: Twenty-five patients were identified by characteristic symptoms (abrupt onset of photopsias, followed by large scotomata at or connected to the blind spot), ocular findings (paucity of pigmentary changes with no sign of vitreous inflammation and abnormal electroretinogram in at least 1 eye), and a negative family history for retinitis pigmentosa. OBSERVATION PROCEDURES: Patients underwent a full comprehensive ophthalmologic examination, fundus retinography, Goldmann kinetic visual field (GVF), and full-field electroretinogram (ffERG). Blood samples were also obtained to verify for the presence of antiretinal antibodies by Western blot analysis. Main OUTCOME MEASURES: Clinical presentation, best-corrected visual acuity (BCVA), fundus abnormalities, visual field defects, ffERG changes, and presence of antiretinal antibodies. Results Sixteen patients (64%) presented with photopsias, 56% (14/25) with night blindness, and 56% (14/25) with loss of peripheral vision. Sixty-four percent (16/25) of cases were bilateral. All patients demonstrated retinal vascular attenuation, optic nerve head pallor, and mottling of retinal pigment epithelium. The most common visual field changes included enlargement and expansion of the blind spot extending into large pericentral or other types of scotomata (64%). Both scotopic and photopic ffERG values were abnormal and affected to a similar degree in our patients. Nine patients (36%) had a greater than 20% asymmetry in ERG values between the 2 eyes. All patients had antiretinal antibodies on Western blot with an average of 6.6 bands. Conclusion Evidence suggests that AZOOR is a unique form of autoimmune retinopathy and retinal manifestation suggests possible antiretinal antibody leakage from the disc margin with spread of immune products under the retina, resulting in large scotomata that connect to the optic nerve head.

AB - Purpose To perform a comprehensive review and to investigate the presence and role of autoimmune antibodies in 25 cases of acute zonal occult outer retinopathy (AZOOR) identified using the classification originally proposed by J. Donald Gass. Design Observational case series. Methods SETTING: Institutional. STUDY POPULATION: Twenty-five patients were identified by characteristic symptoms (abrupt onset of photopsias, followed by large scotomata at or connected to the blind spot), ocular findings (paucity of pigmentary changes with no sign of vitreous inflammation and abnormal electroretinogram in at least 1 eye), and a negative family history for retinitis pigmentosa. OBSERVATION PROCEDURES: Patients underwent a full comprehensive ophthalmologic examination, fundus retinography, Goldmann kinetic visual field (GVF), and full-field electroretinogram (ffERG). Blood samples were also obtained to verify for the presence of antiretinal antibodies by Western blot analysis. Main OUTCOME MEASURES: Clinical presentation, best-corrected visual acuity (BCVA), fundus abnormalities, visual field defects, ffERG changes, and presence of antiretinal antibodies. Results Sixteen patients (64%) presented with photopsias, 56% (14/25) with night blindness, and 56% (14/25) with loss of peripheral vision. Sixty-four percent (16/25) of cases were bilateral. All patients demonstrated retinal vascular attenuation, optic nerve head pallor, and mottling of retinal pigment epithelium. The most common visual field changes included enlargement and expansion of the blind spot extending into large pericentral or other types of scotomata (64%). Both scotopic and photopic ffERG values were abnormal and affected to a similar degree in our patients. Nine patients (36%) had a greater than 20% asymmetry in ERG values between the 2 eyes. All patients had antiretinal antibodies on Western blot with an average of 6.6 bands. Conclusion Evidence suggests that AZOOR is a unique form of autoimmune retinopathy and retinal manifestation suggests possible antiretinal antibody leakage from the disc margin with spread of immune products under the retina, resulting in large scotomata that connect to the optic nerve head.

UR - http://www.scopus.com/inward/record.url?scp=85013177875&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85013177875&partnerID=8YFLogxK

U2 - 10.1016/j.ajo.2016.12.001

DO - 10.1016/j.ajo.2016.12.001

M3 - Article

C2 - 27993590

AN - SCOPUS:85013177875

VL - 176

SP - 210

EP - 218

JO - American Journal of Ophthalmology

JF - American Journal of Ophthalmology

SN - 0002-9394

ER -