Purpose: To report a case of acute Vogt-Koyanagi-Harada (VKH) disease with unilateral clinical manifestations followed by late fellow eye involvement. Case Report: This case report reviews the 12-month follow-up observation of a 44-year old woman who presented to the emergency department with unilateral progressive and painless visual blurring. Ophthalmoscopic findings, best-corrected visual acuity (BCVA), fluorescein angiography (FAG), enhanced-depth optical coherence tomography (EDI-OCT), indocyanine green angiography, and response to treatment were evaluated. Her BCVA was 20/50 (logMAR: 0.4) in the right eye and 20/20 (logMAR: 0) in the left eye. Eye examination revealed optic disc swelling and multiple serous retinal detachments in the right eye and a normal left eye. She had headache, dysacusia, and mild hearing problem. Her past ocular and drug histories were unremarkable. Retinal imaging revealed characteristic features of VKH in the right eye. All laboratory testing results were inconclusive. VA and OCT findings significantly improved following the treatment with methylprednisolone 1 g/day continued by tapering dose of oral prednisolone. Two months after the presentation and during prednisolone tapering, VA of the left eye decreased and fundus examination revealed multiple serous retinal detachments in this eye. Conclusion: Ophthalmologists should recognize unilateral and asymmetrical VKH disease with subtle systemic involvement. Â© 2020 JOURNAL OF OPHTHALMIC AND VISION RESEARCH | PUBLISHED BY KNOWLEDGE 

Dourandish, M.

Hosseini, S.M.

Mazouchi, M.

English

kashan university of medical sciences

Case Report
Unilateral Presentation of Vogt-Koyanagi-Harada
Syndrome
Seyedeh Maryam Hosseini1, MD; Maryam Dourandish1, MD; Marjan Mazouchi2, MD
1Eye Research Center, Mashhad University of Medical Sciences, Mashhad, Iran
2Eye Research Center, Matini Eye Hospital, Kashan University of Medical Sciences, Kashan, Iran
ORCID:
Seyedeh Maryam Hosseini: https://orcid.org/0000-0001-9804-0390
Maryam Dourandish: https://orcid.org/0000-0001-9804-0390
Abstract
Purpose: To report a case of acute Vogt-Koyanagi-Harada (VKH) disease with unilateral
clinical manifestations followed by late fellow eye involvement.
Case Report: This case report reviews the 12-month follow-up observation of a 44-year
old womanwho presented to the emergency department with unilateral progressive and
painless visual blurring. Ophthalmoscopic findings, best-corrected visual acuity (BCVA),
fluorescein angiography (FAG), enhanced-depth optical coherence tomography (EDI-
OCT), indocyanine green angiography, and response to treatment were evaluated. Her
BCVA was 20/50 (logMAR: 0.4) in the right eye and 20/20 (logMAR: 0) in the left eye.
Eye examination revealed optic disc swelling and multiple serous retinal detachments
in the right eye and a normal left eye. She had headache, dysacusia, and mild
hearing problem. Her past ocular and drug histories were unremarkable. Retinal imaging
revealed characteristic features of VKH in the right eye. All laboratory testing results
were inconclusive. VA and OCT findings significantly improved following the treatment
with methylprednisolone 1 g/day continued by tapering dose of oral prednisolone. Two
months after the presentation and during prednisolone tapering, VA of the left eye
decreased and fundus examination revealed multiple serous retinal detachments in this
eye.
Conclusion: Ophthalmologists should recognize unilateral and asymmetrical VKH
disease with subtle systemic involvement.
Keywords: Asymmetric VKH; Enhanced-Depth Optical Coherence Tomography; Indocyanine
Green Angiography; Vogt-Koyanagi-Harada Disease
J Ophthalmic Vis Res 2020; 15 (1): 99–103
Correspondence to:
Maryam Dourandish, MD. Eye Research Center, Khatam-
al-Anbia Eye Hospital, Abutaleb Junction, Gharani Blvd.,
Mashhad University of Medical Sciences, Mashhad
91959, Iran.
E-mail: Maryam.dourandeesh.dl@gmail.com
Received: 05-02-2018 Accepted: 20-08-2018
Access this article online
Website: https://knepublishing.com/index.php/JOVR
DOI: 10.18502/jovr.v15i1.5954
INTRODUCTION
Vogt-Koyanagi-Harada disease (VKH) is a rare,
chronic, inflammatory granulomatous multisystem
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How to cite this article: Hosseini SM, Dourandish M, Mazouchi M. Vogt-
Koyanagi-Harada with Unilateral Clinical Presentation: A Case Report. J
Ophthalmic Vis Res 2020;15:99–103.
© 2020 JOURNAL OF OPHTHALMIC AND VISION RESEARCH | PUBLISHED BY KNOWLEDGE E 99
A Case of Unilateral VKH Syndrome; Hosseini et al
disorder of unknown cause that is characterized by
bilateral panuveitis, iridocyclitis, exudative retinal
detachment, and optic disc swelling. The disorder
affects melanocyte-rich tissues, including the eyes,
auditory system, meninges, skin, and hair.[1–5]
The diagnosis of VKH is based on the Revised
Diagnostic Criteria (RDC) of 2001.[6, 7] The disease
is classified into three categories: complete, incom-
plete, and probable VKH.[6]
The disease is divided into four consecutive
stages, namely prodromic, acute uveitic, convales-
cent, and chronic.[7] Approximately 17–73%of cases
progress to chronic stage.[8] It usually affects both
eyes simultaneously.[1, 6] However, unilateral ocular
involvement at onset, followed by later involvement
of the second eye in 2–3 weeks is reported in 30%
of the cases.[7, 9] Reports of long-term follow-up in
patients with unilateral ocular disease are few.[8–11]
Ocular imaging modalities, including fluores-
cein (FA) and indocyanine angiography (ICGA);
optical coherence tomography (OCT), especially
enhanced-depth imaging OCT (EDI-OCT); and
ultrasonographic findings are useful in the diagno-
sis and follow-up of patients.[6]
Many reports have indicated that patients may
not manifest all diagnostic criteria but seem to have
the disease.[1] Herein, we report an interesting case
of clinically unilateral probable VKHwith subclinical
fellow eye involvement on ICGA, which progressed
to clinical involvement later.
CASE PRESENTATION
A 44-year-old woman with progressive and pain-
less visual blurring of the right eye for two
days presented to the emergency department
of the Khatam-al-Anbia Eye Hospital, affiliated
to the Mashhad University of Medical Sciences,
Mashhad, Iran. She had no history of ocular
surgery or trauma. Her medical, ocular, and drug
histories were unremarkable. The best-corrected
visual acuities (BCVAs) of the right and left eyes
were 20/50 (logMAR: 0.4) and 20/20 (logMAR: 0),
respectively. Her slit-lamp examination result was
normal. Intraocular pressure was 14 mm Hg in
both eyes. Fundus examination revealed optic disc
swelling and multiple serous retinal detachments
in the right eye and normal left retina. Retinal
imaging revealed characteristic features of VKH in
the right eye. FA showed focal areas of delayed
choroidal perfusion, multifocal areas of pinpoint
leakage, pooling, and optic nerve staining (Figures
1 and 2). ICGA disclosed early hyper-cyanescence
of the stromal choroidal vessels, which led to
late hyper-cyanescence in both eyes and multiple
hypofluorescent dark dots in the right eye [Figure 3
(A andB)]. The EDI-OCT findings included choroidal
thickening, loculated spaces of the subretinal fluid
with bands, high macular retinal detachment, sub-
retinal membranous structure, and hyper-reflective
dots in subretinal fluid [Figure 4 (A and B)]. Results
of laboratory tests, including complete blood cell
count (CBC), sedimentation rate, C-reactive protein,
syphilis serology, toxoplasma serology, PPD (puri-
fied protein derivative) skin test, hepatitis serol-
ogy, and autoimmunity markers (ANA [Antinuclear
Antibody], RF [rheumatoid factor], ACE [angiotensin
converting enzyme]), and chest radiography were
negative.
The initial diagnosis was VKH with unilateral
clinical presentation and subclinical involvement of
the left eye disclosed on ICGA. The patient was
treated with intravenous high-dose corticosteroid
(pulse methylprednisolone 1 g/day) followed by
oral prednisolone (1 mg/kg). VA and OCT find-
ings significantly improved. Two months later and
during rapid tapering of the corticosteroid dose
due to systemic complications, VA of the left eye
decreased, associated with anterior chamber and
vitreous cellular reaction. The notable finding was
the presence of vitreous and anterior chamber cells
in the left eye, which was very different from the
condition of the right eye in the first presentation
with an absence of cellular reaction in anterior
chamber and vitreous. Fundoscopic examination
revealed multiple serous retinal detachments. Slit-
lamp examination and fundoscopy of the right eye
had normal results [Figure 3 (C and D), Figure 4 (C
and D)]. Therefore, the dose of prednisolone was
increased, and methotrexate (MTX) 12.5 mg/week
was added. VA, clinical and paraclinical exam-
ination results significantly improved afterwards.
The disease was in complete remission 12 months
after treatment with MTX and tapering of the
prednisolone [Figure 4 (E and F)].
DISCUSSION
VKH typically presents as a bilateral symmet-
ric granulomatous uveitis. Here, we introduce a
case with unilateral clinical presentation, who had
abnormalities in ICGA of both eyes. This implies the
100 JOURNAL OF OPHTHALMIC AND VISION RESEARCH VOLUME 15, ISSUE 1, JANUARY-MARCH 2020
A Case of Unilateral VKH Syndrome; Hosseini et al
Figure 1. Fundus auto-fluorescence (FAF). (A) Hypo-autofluorescence area in macular area and hyper-auto-fluorescence ring in
inferior of macula in the right eye. (B) Normal FAF in the left eye.
Figure 2. Fluorescein angiography at presentation (arteriovenous phase). (A) Multifocal areas of pinpoint leakage (blue arrow),
pooling (yellow arrows) staining (right eye ). (B) Normal in the left eye.
importance of ICGA in the diagnosis and treatment
of VKH.
VKH is diagnosed based on clinical and angio-
graphic findings. Although according to the cur-
rent revised diagnostic criteria, bilateral ocular
involvement is necessary for the diagnosis of VKH,
there are a few reports of unilateral or asym-
metrical ocular involvement.[9, 10, 12, 13] Our patient
had clinical and paraclinical features compatible
with VKH except for unilaterality at initial presen-
tation.
In this patient, except for headache, dysacu-
sia, and mild hearing loss, review of systems
and laboratory tests were normal. Ophthalmic
examination, FAG, and ICGA findings were typ-
ical of VKH: serous retinal detachment, focal
areas of delayed choroidal perfusion, multifocal
areas of pinpoint leakage, pooling, and optic
nerve staining on FAG; hypofluorescent dark
dots on ICGA; and choroidal thickening, locu-
lated spaces of subretinal fluids with bands, high
macular retinal detachment, subretinal membra-
nous structure, and hyper-reflective dots on SD-
OCT. The differential diagnosis of VKH includes
infectious diseases (bacteria, fungal, tuberculosis,
and syphilis), malignancies (intraocular lymphoma,
diffuse uveal lymphoid hyperplasia, systemic lym-
phoma, or leukemia), and inflammatory diseases
(posterior scleritis, sarcoidosis, acute posteriormul-
tifocal placoid pigment epitheliopathy, and multiple
evanescent white dot syndrome choroidopathy).
These diseases can be excluded on the basis
JOURNAL OF OPHTHALMIC AND VISION RESEARCH VOLUME 15, ISSUE 1, JANUARY-MARCH 2020 101
A Case of Unilateral VKH Syndrome; Hosseini et al
Figure 3. Indocyanine Green angiography at presentation. (A) Early phase of the right eye reveals multiple hypo-fluorescent dark
dots in posterior pole that remains hypofluorescent until late phase (blue arrow). (B) No abnormal fluorescence in early and late
phase in left eye except for mild dilation of choroidal vessels (yellow arrows). (C, D) Two months after the disease presentation
during the tapering of corticosteroid: hypofluorescent dark dots in both eyes (green arrows) implying still active disease in the
right eye and involvement of the left eye.
of clinical manifestations, disease course, sys-
temic medical workup, and ophthalmic imaging
findings.[9, 10, 12, 13]
There was a significant improvement in BCVA
and OCT findings in this case following the
administration of high-dose intravenous corticos-
teroid. During the rapid tapering of the cor-
ticosteroid due to systemic complications, fel-
low eye involvement appeared clinically. Increas-
ing the dose of corticosteroid and addition of
MTX caused visual acuity and fundus features
to improve markedly. High-dose corticosteroid
is the mainstay treatment of VKH. Treatment
must be continued with gradual tapering of
the oral corticosteroid over at least six months,
along with introduction of corticosteroid-sparing
agents such as MTX, mycophenolate mofetil, and
cyclosporine A. Poor responders to corticosteroids
and other immunomodulatory agents would ben-
efit from biological agents such as rituximab and
infliximab.[8–10, 13]
In summary, VKH can present with atypical
features such as unilateral or asymmetrical signs
and symptoms. Meticulous diagnosis is essen-
tial for prompt initiation of corticosteroids and
immunomodulatory agents, as aggressive and
rapid initiation of treatment is necessary to control
the inflammation and thereby prevent the devel-
opment of sequela and visual disability. Imaging
modalities, especially ICGA, can be invaluable
102 JOURNAL OF OPHTHALMIC AND VISION RESEARCH VOLUME 15, ISSUE 1, JANUARY-MARCH 2020
A Case of Unilateral VKH Syndrome; Hosseini et al
Figure 4. Spectral domain optical coherence tomography SD-OCT at presentation. (A) Large intraretinal cyst (red asterisk), high
macular retinal detachment (blue arrow), subretinal hyperreflective membranous structure (yellow arrow), high reflective dots
(green arrow). (B) Left eye: normal. (C) Complete resolution of intraretinal cyst and subretinal hyperreflectivemembranous structure
(D) but involvement of the left eye inmonth 2 during the tapering of the treatment. (E, F) Normal SD-OCT of two eyes after adequate
treatment.
in the diagnosis of VKH with unilateral presen-
tation, which shows subtle subclinical involve-
ment.
Financial Support and Sponsorship
Nil.
Conflicts of Interest
There are no conflicts of interest.
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Unilateral presentation of Vogt-Koyanagi-Harada syndrome

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Unilateral presentation of Vogt-Koyanagi-Harada syndrome

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