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The clinical spectrum of Vogt-Koyanagi-Harada syndrome: A single-center experience from South India

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Aim To report the clinical features, investigations, management, and outcome in typical cases of Vogt-Koyanagi-Harada Syndrome (VKH) in a tertiary eye care center in South India. Materials and methods Retrospective interventional case series of VKH patients. Results Seventy-one eyes of 36 patients (age range 12-68 years) were included. Anatomical diagnoses were posterior uveitis (15 eyes) and pan uveitis (56 eyes). The classification of the cases with regard to VKH disease was as follows: six eyes were classified as complete, 41 eyes as incomplete, and 24 eyes as probable VKH disease. Commonest extraocular manifestation noted in 25 cases was headache. The more common ocular presentations were disc hyperemia (40 eyes) and exudative retinal detachment in the posterior pole (46 eyes) and in the retinal periphery (24 eyes). All patients were managed on systemic steroids. Systemic immunosuppressive therapy was given in 21 cases. Majority of the participants had good visual outcome. Conclusion Early recognition and aggressive treatment of VKH disease result in good visual outcome in typical VKH cases.
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Internet Journal of Rheumatology and Clinical Immunology Page 1 of 8
IJRCI. 2013;1(S1):SO2
ORIGINAL ARTICLES
The clinical spectrum of Vogt-Koyanagi-Harada syndrome: A single-center
experience from South India
Padmamalini Mahendradas1*, Ankush Kawali2, Neha Bharti3, Dhawal Haria4, Poornachandra Gowda3, Naren Shetty4,
Rohit Shetty5, Bhujang K Shetty6
1Head of Department of Uveitis and Ocular Immunology, Narayana Nethralaya and Postgraduate Institute of Ophthalmology, 21/C, Chord road,
Rajajinagar, Bangalore
2 Consultant, Department of Uveitis and Ocular Immunology, Narayana Nethralaya and Postgraduate Institute of Ophthalmology
3 Fellow, Vitreo-Retina Services, Narayana Nethralaya and Postgraduate Institute of Ophthalmology
4 Postgraduate, Narayana Nethralaya and Postgraduate Institute of Ophthalmology
5 Vice Chairman, Narayana Nethralaya and Postgraduate Institute of Ophthalmology
Abstract
Aim
To report the clinical features, invesgaons, management, and outcome in typical cases of Vogt-Koyanagi-Harada
Syndrome (VKH) in a terary eye care center in South India.
Materials and methods
Retrospecve intervenonal case series of VKH paents.
Results
Seventy-one eyes of 36 paents (age range 12-68 years) were included. Anatomical diagnoses were posterior uveis
(15 eyes) and pan uveis (56 eyes). The classicaon of the cases with regard to VKH disease was as follows: six eyes
were classied as complete, 41 eyes as incomplete, and 24 eyes as probable VKH disease. Commonest extraocular
manifestaon noted in 25 cases was headache. The more common ocular presentaons were disc hyperemia (40 eyes)
and exudave renal detachment in the posterior pole (46 eyes) and in the renal periphery (24 eyes). All paents were
managed on systemic steroids. Systemic immunosuppressive therapy was given in 21 cases. Majority of the parcipants
had good visual outcome.
Conclusion
Early recognion and aggressive treatment of VKH disease result in good visual outcome in typical VKH cases.
Introduction
Vogt-Koyanagi-Harada syndrome (VKH) is an oculo-
cutaneo-meningeal syndrome characterized by
granulomatous panuveitis associated with neurological,
auditory, and cutaneous manifestations. It is an autoimmune
disorder directed against melanocytes in the uveal tract,
meninges, inner ear, and skin. The disease is commonly
seen in pigmented races. Asians, Native Americans,
Hispanics, Asian Indians and those with Middle Eastern
heritage are most frequently affected, but interestingly the
disease is rare among Sub-Saharan Africans, indicating
that the amount of skin pigmentation alone is not the sole
etiologic factor in the pathogenesis of VKH syndrome.1
Identication of association with major histocompatibility
complex class II antigens, strongly supports an underlying
genetic predisposition in the pathogenesis of the disorder.
In Japan, VKH disease accounts for more than 8% of
uveitis, 2.9% in the Middle East, 1.2% in Europe and 1%
to 4% in the United States.2, 3 In a study done at one of the
referral eye care centers in South India, the prevalence of
VKH in uveitis cohorts was found to be 1.4% to 3.5%.4
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Though ocular manifestations are common, VKH patients
frequently land up in general physician’s clinic or they are
referred to neurologist for their extra-ocular features like
meningism and undergo unnecessary investigations and
imaging, which may further delay the treatment. The disease
has very good prognosis when managed in time by an
ophthalmologist.
The aim of this study is to analyze demographics, clinical
characteristics, treatment response, and complications in
VKH patients referred to a tertiary eye care center in South India.
Materials and methods
This is a retrospective, non-randomized, non-
comparative interventional case series. Patients
diagnosed as typical VKH disease presenting to the
department of uveitis and ocular immunology between
January 2005 and December 2012 in a tertiary eye care
center in Bangalore, India were included in the study.
Institutional ethical committee approval was obtained for
the retrospective chart review for this study. The clinical
records of 35 patients diagnosed with VKH were reviewed
for patient demographics, clinical signs, investigations,
treatment received, complications, and visual outcome. All
patients underwent a complete ophthalmic examination,
including measurement of best corrected visual acuity
(VA), slit-lamp examination, applanation tonometry,
dilated fundus examination with scleral indentation, colour
fundus photography, and ocular ultrasonography (if the
view of the fundus was not clear). External examination
evaluated the presence of poliosis and vitiligo.
The diagnosis of VKH disease was made according to
the clinical presentations and it was conrmed by fundus
uorescein angiography (FFA) in all cases. Additional
investigations such as B-scan ultrasonography, spectral
domain optical coherence tomography (Spectralis,
Hiedelberg engineering) and indocyanine green angiography
(ICG) was done in selected cases. A history of trauma was
ruled out in all cases. Investigations were done to rule out
tuberculosis (Mantoux skin hypersensitivity test, PA view of
chest X-ray), syphilis (Treponema pallidum hemaglutination)
and sarcoidosis (serum angiotensin converting enzyme).
We used revised diagnostic criteria for Vogt-Koyanagi-
Harada disease by Read et al. for classifying the study
participants into complete, incomplete, and probable VKH.5
All patients with associated diseases such as tuberculosis,
sarcoidosis, syphilis or trauma were excluded. All
the patients were treated with systemic corticosteroid
therapy. Topical corticosteroids and immunosuppressive
therapy were used in selected cases. Statistical
analysis was done using the SPSS version 12 software.
Results
Thirty-ve patients were included in the study. Age group
of the subjects ranged between 12-68 years (mean 34.1
years). Eleven patients were male and 24 patients were
female. Thirty-four patients had bilateral presentation
and 1 patient had unilateral VKH. The clinical type of
VKH disease was classied as complete in 3 patients,
incomplete in 20 patients, and probable in 12 patients.
Clinical presentation was posterior uveitis in 15 eyes
and panuveitis in 55 eyes. Thirty eyes (42.8%) had acute
VKH disease, 28 eyes (40%) had chronic presentation,
and 12 eyes (17.1%) had recurrent uveitis. (Table 1)
Meningism and headache were the commonest acute
presentations followed by poliosis and vitiligo in chronic
cases. Sensorineural hearing loss was present in 2 cases
(Table 2). Eighteen eyes presented with best corrected
visual acuity of 20/200 or worse at presentation (Fig 5).
The ocular ndings of slit-lamp examination were
circumciliary congestion in 38 eyes (54.2%), keratic
precipitates in 34 eyes (49.2%) and iris nodules in 12
eyes (17.1%) with the presence of anterior chamber
are and cells (Table 3). Fundus examination revealed
vitritis in 70 eyes (100%), optic disc involvement in 40
eyes (57.1%), exudative retinal detachment in 45 eyes
(64.2%), choroidal detachment in 5 eyes (7.1%), sunset
glow fundus in 39 eyes (55.7%), and Dalen-Fuchs
nodules in 20 eyes (28.5%) (Table 3). FFA was done
in all patients, which revealed multiple hyperuorescent
dots at the level of retinal pigment epithelium in 30 eyes,
optic disc leakage in 55 eyes, and late accumulation of
uorescein in the subretinal space in 31 eyes. The ICG
showed early hypouorescent spots, diffuse choroidal
hyperuorescence, and choroidal perivascular leakage
in 16 cases. Follow-up ICG conducted after 6-8 weeks
revealed multiple hypouorescent lesions, even when the
fundoscopic examination and FFA were unremarkable in
13 cases. Low- to medium- reective diffuse thickening of
the choroid was demonstrated by 20 MHz ultrasonography
and was most marked in the juxtapapillary region. This
was associated with retinal detachments in 45 eyes
and choroidal detachments in ve acute cases. Optical
coherence tomography (OCT) ndings in VKH in active
state were multi-lobular serous retinal detachments,
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intra-retinal edema, subretinal septae, and dot
reex in subretinal space. Dalen-Fuchs nodules and
retinal pigment epithelium (RPE) and choriocapillary
hypertrophy were noted in chronic VKH cases.
Treatment duration ranged from 6 to 36 months (mean18
months). Patients with anterior segment inammation
were treated with topical corticosteroids and cycloplegic
agents, in addition to systemic immunosuppressive
therapy. Two patients who could not tolerate the systemic
immunosuppressive therapy due to transient increase
in liver enzymes were treated with bilateral periocular
posterior subtenon’s triamcinolone acetonide injection
and subsequently with systemic immunosuppressive
therapy. Intravenous methyl prednisolone 1gm once
daily for three days was given to 17 patients (48.5%)
who had serous retinal detachment. Oral prednisolone
acetate was given to all the patients (Table 4). The
median duration of oral prednisolone was 7 months
(range 6-12 months). Two patients who developed
diabetes mellitus during the course of the disease
switched over to deozacort along with azathioprine.
Oral immunosuppressives were used for a median
duration of 14 months (range 6-30 months) in 22 patients
(62.81%). Drugs such as methotrexate (14 patients),
azathioprine (11 patients), mycophenolate mofetil (2
patients) and cyclosporine (1 patient) were used (Table
4). Six patients received two immunosuppressive drugs
and triple immunosuppressive therapy comprising of
mycophenolate mofetil and cyclosporine along with
tapering dose of oral steroids by one patient. Once the
ocular inammation and retinal detachments had resolved,
the fundus changes showed near normal fundus in 5
patients and pigmentary changes with sunset glow fundus
in 15 patients of acute uveitis and all patients of chronic
and recurrent uveitis. Recurrences were seen in 12 eyes
(panuveitis in 8 eyes and posterior uveitis in 4 eyes).
Table 1: Demographic and clinical characteristics of VKH patients
Demographic variables No. (%)
Mean Age (yrs) 34.1 (11- 68)
Gender
Male
Female
11(31.4%)
24 (68.5%)
Laterality
Bilateral 35 (100%)
Anatomical classication
Panuveitis
Posterior
55 eyes (78.9%)
15 eyes (21.1%)
Clinical types
Probable
Incomplete
Complete
24 eyes / 12 patients (34.2%)
40 eyes / 20 patients (57.1%)
6 eyes / 3 patients (8.5)
Course
Acute
Chronic
Recurrent
30 eyes (42.8%)
28 eyes (40%)
12 eyes (17.1%)
Table 2: Incidence of diverse systemic manifestations
Systemic manifestations No. (%)
Meningismus
Headache
Poliosis
Vitiligo
Hearing loss
Tinnitus
20 (57.1)
20 (57.1)
8 (11.4)
12 (34.2)
2 (5.7)
14 (40)
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Anterior segment manifestations No. (%)
Circumciliary congestion 38 eyes (54.2)
Keratic precipitates (KP)
Fine KPs
Mutton fat KPs
34 eyes (49.2)
24 eyes (34.2)
10 eyes (14.2)
Iris nodules 12 eyes (17.1)
Flare
1+
2+
3+
45 eyes (64.2)
23 eyes (32.8)
19 eyes (27.1)
3 eyes (4.2 )
Cells
1+
2+
3+
52 eyes (74.2)
24 eyes (34.2)
25 eyes (35.7)
3 eyes (4.2)
Posterior segment manifestations No. (%)
Vitritis
1+
2+
3+
70 eyes (100)
52 eyes (74.2)
16 eyes (22.8)
2 eyes (2.8)
Disc hyperemia 40 eyes (57.1)
Disc edema 40 eyes (57.1)
Retinal detachment
Posterior pole
Periphery
45 eyes (64.2)
45 eyes
24 eyes
Sunset glow fundus 39 eyes (55.7)
Dalen-Fuchs nodules 20 eyes (28.5)
Choroidal detachment 5 eyes (7.1)
Table 3: Clinical ndings on anterior and posterior segment manifestations
Drugs used No. (%)
Topical corticosteroids 61 eyes (87.1)
Oral steroids 35 patients (100)
Periocular steroids 2 patients (5.7)
Intravenous methyl prednisolone 17 patients (48.5)
Immunosuppressive 22 patients (62.8)
Methotrexate 14 patients (40)
Azathioprine 11 patients (31.4)
Cyclosporine 1 patient (2.8)
Mycophenolate mofetil 2 patients (5.7)
Patients on more than one immunosuppressives 6/22 patients (17.1)
Patients on more than two immunosuppressives 1/22 patient (2.8)
Table 4: Medical management of VKH syndrome
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Figures 1- 4 indicate illustrative clinical ndings observed in VKH patients
Figures 1A - 1C : Extraocular manifestations in chronic VKH syndrome
Figures 2A, 2B: Montage colour fundus photograph of the right eye (Figure 2A) and left eye (Figure 2B) showing the hyperemic disc, multiple serous
retinal detachments in the posterior pole with inferior exudative retinal detachments. Patient was treated with IVMP followed by oral steroids and
systemic methotrexate therapy. The patient further presented with recurrence of inammation after stopping the systemic immunosuppressive therapy
on her own. Subsequently, she was treated with systemic steroids and methotrexate therapy. Follow-up picture 2C and 2D reveals sunset glow fundus
with subretinal brosis.
Figure 3: Chronic VKH-fundus photograph showing sunset glow fundus with Dalen-Fuchs nodules in the left eye. FFA & ICG: Montage image showing
normal uorescein angiogram with hypouorescent dark spots in the indocyanine angiography suggestive of active lesions in the choroid.
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Figure 4: A 21-year- old male diagnosed to have bilateral exudative retinal detachment (Figure 4A, 4B) with multiple pinpoint hyperuorescence
with pooling of the dye (Figure 4C, 4D) treated with triple immunosuppressive therapy, showing complete resolution of the inammation in both eyes
(Figure 4E, 4F).
Visual outcome
In our series of patients, a better than or equal to
20/40 VA was maintained in 54 eyes, while 5 eyes had
poor visual outcome due to cataract formation (Fig 5).
Complications
Complicated cataract, seen in 20 eyes, was the commonest
ocular complication. Increased intraocular pressure in 8
eyes and hypotony and subretinal brosis in 2 eyes (Table
6) were also noted. Extraocular manifestations were seen
in chronic and recurrent cases. Two patient developed
urinary tract infection due to E. Coli infection and one patient
developed pneumonia. All the three patients were managed
with appropriate systemic antibiotic therapy. Transient
increase in liver enzymes was observed in 2 patients.
Fig 5: Visual acuity at presentation and at nal follow-up
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Discussion
Vogt-Koyanagi-Harada disease (VKH), a multisystem
autoimmune disorder targeting predominantly
melanocytes, affects organs such as eye, skin, inner ear
and meninges. In the eye, the disease affects the uveal
tract, manifesting as granulomatous panuveitis. Acute
presentation is characterized by the presence of exudative
retinal detachment with preservation of the choriocapillaris.
In the chronic stage, the disease is characterized by the
presence of Dalen-Fuchs nodules and focal chorioretinal
atrophy with loss of retinal pigment epithelium.6 In chronic
stage, patients develop extraocular manifestations. The
ndings from our study as well as from the earlier reports
show that women are more likely to develop VKH disease
than men, whereas Soon-Phaik Chee et al. have reported
equal distribution in both sexes.7-10, 11 The observations in
elderly patients were similar to the study by Kiyomoto et al.12
The present study ndings are similar to that of Ilknur
Tugal-Tutkun et al. With regard to clinical types of the
disease, incomplete VKH (57.7%) was identied as the
commonest followed by probable (33.8%) and complete
forms (8.4%) as published by Ilknur Tugal-Tutkun et al.13
Extra-ocular manifestations were seen in chronic cases
and the results were similar to hispanic population.14
Whereas these manifestations are more common in
studies involving Japanese patients.12 The early and
aggressive use of corticosteroids in acute VKH disease
prevented the appearance of the complete spectrum of
the disease in the current study, as shown by Rajendram
et al.15 Systemic steroid therapy was given for longer
periods of time along with immunosuppressive therapy.
The diagnosis of VKH disease is based on history and
clinical ndings with supportive evidence from ancillary
tests, including fundus uorescein angiography, which
showed classical presentation such as multiple pin-point
hyperuorescence in the early phase at the level of the
RPE, with classical pooling of the dye in the late phase
in all acute presentations. ICG angiography showed
more extensive areas of involvement characterized
by staining and leakage of the larger choroidal
vessels with multiple hypouorescent dark spots.16, 17
In chronic cases, even in the absence of inammatory activity
on fundus examination and uorescein angiography, ICGA
showed the presence of multiple hypouorescent dots
suggestive of active inammation in the choroid, which
helped us to continue the systemic immunosuppressive
therapy. OCT was used when the media was clear to
document the changes and B scan was used in all acute
cases and also in cases of hazy media, where we could not
visualize the fundus to monitor the response to treatment.
All our patients were treated with systemic steroid
therapy. Periocular steroid injections were given in 2
eyes when the systemic immunsupppressive therapy
was contraindicated due to systemic infection. Systemic
immunosuppressive therapy was given to patients who
needed prolonged high-dose corticosteroids and also in
chronic recurrent disease. Systemic immunosuppressive
therapy was administered to 61.1% of the patients.
Double immunosuppressive therapy was given in 27.3%
cases. Triple immunosuppressive therapy (4.5%) with
mycophoelate mofetil, cyclosporine and systemic steroids
were given to one patient and it resulted in good control of
inammation with good visual recovery. Further prospective
evaluation of the role of immunomodulatory agents in the
treatment of VKH disease is recommended due to small
cohort size and retrospective nature of the present study.
CSF analysis was not done in majority of the cases
and hence the probability of CNS being not involved
is primarily due to the absence of clinical signs and
symptoms. Other limitations of the present study were
non-inclusion of atypical presentations and small study
cohort size. We found that the most frequent ocular
complication of VKH disease was cataract (20 eyes,
28.1%), which is similar to other earlier studies. 5, 18, 19
Further multicentric prospective studies to
explore role of immunomodulatory agents in the
treatment of VKH disease are recommended.
In summary, the clinical features of VKH in South India
are similar to the earlier reports, except for the increased
presentations of posterior segment inammations than the
anterior segment reactions during the recurrences.8 Extra
ocular manifestations were not noticed in acute cases
with aggressive management with systemic steroids and
immunosuppressive therapy. Chronic and recurrent cases
had extraocular manifestations at the time of presentation
in majority of the cases. Early and aggressive therapy
resulted in good visual outcome in majority of our patients.
Acknowledgement
We acknowledge the contribution of the treating physicians (Dr. Keerthy
Shetty and Dr. Shylaja Shyamsundar) and the immunologists (Dr.
Chandrasekara, Dr. Dharmanand, Dr. Mahendranath, Dr. Ramesh Jois
and Dr. Sathish Kalange) for helping us with the systemic management.
Internet Journal of Rheumatology and Clinical Immunology Page 8 of 8
Competing interests
The authors declare that they have no competing interests.
Citation
Mahendradas P, Kawali A, Bharti N, Haria D, Gowda P, Shetty N, Shetty
R, Shetty BK. The clinical spectrum of Vogt-Koyanagi-Harada syndrome:
A single-center experience from South India. IJRCI. 2013;1(S1):SO2.
Received: 1 August 2013, Accepted: 18 December 2013, Published:
26 December 2013
*Correspondence: Dr Padmamalini Mahendradas,
Head of Department of Uveitis and Ocular Immunology,
Narayana Nethralaya,121/C, Chord road, Rajajinagar, Bangalore
m.padmamalini@gmail.com
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... patients (34 eyes), and probable in 11(36.7%) patients (22 eyes [15]; thirty eyes (42.8%) had acute VKH disease, 28 eyes (40%) had chronic presentation, and 12 eyes (17.1%) had recurrent uveitis [15]. The findings of this present study were also similar to that of Ilknur Tugal-Tutkun et al. with regard to clinical types of the disease; where incomplete VKH (57.7%) was identified as the commonest followed by probable (33.8%) and complete forms (8.4%) [16]. ...
... patients (34 eyes), and probable in 11(36.7%) patients (22 eyes [15]; thirty eyes (42.8%) had acute VKH disease, 28 eyes (40%) had chronic presentation, and 12 eyes (17.1%) had recurrent uveitis [15]. The findings of this present study were also similar to that of Ilknur Tugal-Tutkun et al. with regard to clinical types of the disease; where incomplete VKH (57.7%) was identified as the commonest followed by probable (33.8%) and complete forms (8.4%) [16]. ...
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Background: Vogt-Koyanagi-Harada (VKH) disease is an autoimmune inflammatory disease characterized by bilateral panuveities affecting the young adults. Approximately 3% of the uveitis patients are VKH patients. Objective: To assess the clinical profile of patients with Vogt-Koyanagi-Harada (VKH) disease. Methods: This cross-sectional observational study was conducted over 30 diagnosed patients of VKH disease in uvea clinic of National Institute of Ophthalmology and Hospital (NIO&H), Dhaka, Bangladesh. Patients were selected according to specific selection criteria. All the relevant data were recorded in a pre-designed data collection sheet. Results were analyzed by statistical tests. Results: The mean age of the study patients was 40.2±6.53 years, range 26-59 years. Out of 30 patients; 9(30%) were male and 21(70%) were female. Male to female ratio was 1:2.3. In this study we found all VKH diseases (100% cases) were in both eyes. The clinical type of VKH disease was classified as complete in 2 patients (4 eyes), incomplete in 17 patients (34 eyes), and probable in 11 patients (22 eyes). Regarding clinical presentation; posterior uveitis was in 14 eyes and panuveitis in 46 eyes. Twenty-six eyes (43.3%) had acute VKH disease, 24 eyes (40%) had convalescent stage, and 10 eyes (16.7%) had recurrent uveitis. Meningism [17(56.7%)] and headache [16(53.3%)] were the commonest acute presentations followed by vitiligo [10(33.3%)] and poliosis [3(10.0%)]. Sensorineural hearing loss was present in 2(6.7%) chronic cases. The ocular findings of slit-lamp examination were circumciliary congestion in 32 eyes (53.3%), keratic precipitates in 30 eyes (50.0%) and iris nodules in 10 eyes (16.7%). Fundus examination revealed that vitritis in 60 eyes (100%), optic disc involvement in 34 eyes (56.7%), exudative retinal detachment in 38 eyes (63.3%), sunset glow fundus in 32 eyes (53.3%), Dalen-Fuchs nodules in 16 eyes (26.7%) and choroidal detachment in 4 eyes (6.7%). We found 71.7% of VKH patients had substantial vision loss. Conclusion: Vogt-Koyanagi-Harada disease is a bilateral, diffuse, granulomatous panuveitis, affects more in females of the middle age. Most of our VKH patients were in the chronic stage of the disease and had substantial vision loss. Early recognition and aggressive treatment of VKH disease result in good visual outcome.
... Choroidal detachment, however, is a rare manifestation of VKHdisease. 1 An intravitreal injection may be complicated by vitreous hemorrhage, inflammation, glaucoma, retinal detachment, endophthalmitis, and panophthalmitis. 2 Choroidal detachment is extremely rare following an intravitreal injection and has been reported following an intravitreal sustained release dexamethasone implant injection. 3 To the best of our knowledge, this is the first report of annular choroidal detachment following an intravitreal injection in a case of VKH disease. ...
... Choroidal detachment in VKH disease has been attributed to choroidal inflammation. 1 Inflammation increases the permeability of choroidal vessels allowing leakage from choroidal vessels into the extravascular space. This leakage can secondarily increase resistance to transscleral outflow, causing fluid to accumulate in the suprachoroidal space leading to choroidal detachment. ...
Article
Purpose: To report a unique case of choroidal detachment following an intravitreal injection in a patient with Vogt-Koyanagi-Harada (VKH) disease. Methods: A 33-year-old male, a known case of chronic VKH disease for 9 years developed an inflammatory choroidal neovascular membrane (CNVM) in the right eye. Following an intravitreal ranibizumab injection, he reported loss of vision 2 days later. Clinical examination revealed hypotony with annular serous choroidal detachment. Results: Following an intensive systemic corticosteroid therapy, there was a complete resolution of choroidal detachment and restoration of visual acuity at 1 week of follow up. The subretinal fluid and cystic spaces due toCNVM also resolved completely. Conclusion: This is the first report of a patient with VKH disease developing choroidal detachment following an uncomplicated intravitreal injection of ranibizumab for an inflammatory CNVM.
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Purpose To describe the demographic pattern, clinical characteristics, treatment modalities, and visual outcome in Vogt–Koyanagi–Harada (VKH) disease patients observed at a tertiary eye care centre in South India. Methods A prospective chart analysis of 38 patients with VKH disease. Results Of the 38 VKH patients, 31 (81.6%) were female, the mean age was 39.8 years, and the median duration of symptoms was 10 days (range: 2 to 180 days). The most common ocular complaint was a defective vision (70 eyes or 92.1%). Extraocular manifestations were headache (13 patients or 34.2%) and tinnitus (4 patients or 10.5%). Twenty-four patients (63.2%) presented with bilateral panuveitis, and 12 patients (31.6%) had posterior uveitis. The most common posterior segment presentation included bilateral serous retinal detachment (100%), disc oedema (37 eyes or 48.7%), and vitritis (31 eyes or 40.8%). All patients (100%) were treated with oral and topical prednisolone, based on the ocular inflammation status, 9 patients (23.7%) required dexamethasone (intramuscular), 39 eyes (51.3%) required posterior subtenon triamcinolone, 16 patients (28.9%) required methotrexate, 1 (2.6%) required cyclophosphamide, 1 (2.6%) required azathioprine. At the final visit, the majority of the patients had statistically significant good visual outcomes. Conclusion Prompt diagnosis and early initiation of treatment with systemic, local steroid and immunosuppressive therapy showed faster recovery with good visual outcome.
Article
Purpose: To report an atypical case of Vogt-Koyanagi-Harada (VKH) disease with retrolental bullous retinal detachment (RD). Methods: A case report. Case: A 67-year-old Indian woman with bilateral, gradual visual loss presented with both eyes light perception, keratic precipitates, 2+ cells and bullous RD, which was retrolental in right eye (RE). Systemic investigations were unremarkable. She received systemic corticosteroids, and underwent pars plana vitrectomy (PPV) in left eye (LE). Intraoperatively, sunset glow with leopard-spot fundus were suggestive of VKH disease. Immunosuppressive therapy was added. At 2 years, vision was 3/60 in RE and 6/36 in LE. The LE retina reattached immediately following surgery, while RE exudative RD resolved very gradually following corticosteroids. Summary: This report illustrates diagnostic as well as therapeutic challenge in VKH disease presenting with retrolental bullous RD. PPV provided a faster anatomical and functional restoration than systemic corticosteroid therapy alone, which has potential adverse effects in the elderly.
Article
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To compare the patterns of uveitis, emphasizing similarities and discrepancies, in the Middle East and Europe. Six articles reporting uveitis patterns from the Middle East including a total of 2,693 cases, and seven articles with a sum of 4,379 cases from Europe were analyzed and patterns in each region were defined and compared. In both regions, uveitis was most commonly seen in the fourth decade of life with anterior uveitis being the most common anatomical form. Idiopathic cases accounted for the majority of anterior and intermediate uveitis; toxoplasmosis was the most frequent entity in posterior uveitis while Behcet's disease and idiopathic forms were the next most common causes in the Middle East and in Europe, respectively. Since patterns of uveitis differ in various geographic regions, discovering these patterns would be helpful for the diagnosis and treatment of this broad category of conditions. This necessitates applying a universal diagnostic classification system to enable accurate comparisons.
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The purpose of this study was to describe the prevalence, clinical characteristics, and causes of vision loss in children with Vogt-Koyanagi-Harada disease seen at a uveitis referral center in South India. Charts of patients with Vogt-Koyanagi-Harada disease examined in the uveitis referral clinic of Aravind Eye Hospital between January 1998 and December 2007 were reviewed. A subset of patients <or=16 years of age was identified, and the clinical characteristics and causes of vision loss were evaluated. Vogt-Koyanagi-Harada disease was diagnosed in 267 of 22,959 patients (1.2%) during the study period. Twenty-two children (8.2%) were identified, including 13 girls (59.1%) and 9 boys (40.9%). Age at presentation ranged from 8 years to 16 years, with a mean and a median of 12.6 and 13.5 years, respectively. The most common complaints were blurred vision (39 eyes, 88.6%) and eye redness (36 eyes, 81.8%). Fifteen (68.5%) children had headaches, 6 (27.3%) developed meningismus and alopecia, 4 (18.2%) developed poliosis and vitiligo, and 3 (13.6%) had tinnitus or dysacusis. Initially, all were treated with oral prednisone, but 12 (54.6%) required methotrexate, 5 (22.7%) required azathioprine, and 2 (9.1%) required cyclophosphamide. Seventy-five percent of eyes had a final visual acuity of >or=20/40, whereas 13.6% had a final visual acuity of <or=20/200. Vogt-Koyanagi-Harada disease is an uncommon cause of uveitis in children. The clinical characteristics of pediatric Vogt-Koyanagi-Harada disease in South India resembled those described in cohorts from other regions. Although children in our cohort tended to do well with prompt diagnosis and treatment, long-term vision loss can occur.
Article
Forty-eight cases of the Vogt-Koyanagi-Harada (VKH) syndrome occurring in patients residing in southern California were reviewed. Thirty-six patients were Hispanic and 12 of other racial groups. Symptoms of meningismus, predominantly headache, were present in 32 (67%) cases, but the other characteristic neurologic symptoms, i.e., tinnitus and dysacusis, were present in only eight (17%) and six (13%) cases, respectively. Dermatologic changes were rare; vitiligo occurred in five (10%) patients, alopecia in six (13%), and poliosis in three (6%). In this patient population, extraocular signs and symptoms of the VKH syndrome, other than headache, were unusual. The ocular manifestations of the VKH syndrome are more constant and include iridocyclitis, vitritis, diffuse swelling of the choroid, serous retinal detachment, and optic disc hyperemia. Procedures that may aid in the diagnosis include lumbar puncture, fluorescein angiography, and standardized echography. The ophthalmologist must be prepared to make this diagnosis and initiate treatment with high-dose systemic steroids based on the typical ocular findings even in the absence of other (extraocular) manifestations of this disease.
Article
We reviewed data from 26 patients with Vogt-Koyanagi-Harada syndrome who presented at the Bascom Palmer Eye Institute between March 1969 and February 1990. Visual outcomes were good, with final visual acuity of better than 20/30 in 29 (66%) of 44 eyes and of worse than 20/400 in only three (7%) of 44 eyes. A poor prognosis was associated with the development of choroidal neovascular membranes or chronic uveitis. All patients were treated with systemic corticosteroids. Corticosteroid therapy averaged 6 months, but was prolonged (48 months) in patients who developed chronic uveitis. Disease recurred in nine (43%) of 21 patients in the first 3 months, usually in association with a rapid tapering of steroid dosage. We recommend the early, aggressive use of systemic corticosteroids in patients with Vogt-Koyanagi-Harada syndrome and a gradual tapering of drug dosage for 6 months after presentation.
Article
A retrospective analysis of 33 patients with Vogt-Koyanagi-Harada disease (VKH disease) seen in São Paulo, Brazil, from 1976 to 1985 at a uveitis referral clinic revealed that VKH disease represents 2.5% of the total uveitis cases seen. All cases were bilateral, 30% being men and 70% women. The ethnic distribution was the following: 60% white (with variable Indian or black extraction), 24% darkly pigmented, 9% Orientals (Sansei, third-generation Japanese) and 6% black. The frequency among Orientals was 7 times higher than what would be expected according to the relative frequency of Japanese in the Brazilian population. The age distribution at the onset of the disease was as follows: 12% less than 20 years of age, 60% between 20 and 40 years of age and 27% over 40 years of age. The disease was classified into 3 types with variable extraocular signs. Type I disease was present in 24% of the patients, type II in 51% and type III disease in 24% of the patients. Cataract was present in 40% of the cases and glaucoma was present in 9%. No correlation was found between sex, age at onset, race, type of extraocular involvement and number of extraocular manifestations in considering either visual status or visual prognosis. All patients were treated with systemic steroids. Most of them also received cytotoxic immunosuppressive agents. In this uncontrolled clinical study cytotoxic drug-treated patients seemed to have a better clinical course.
Article
The Vogt-Koyanagi-Harada syndrome (VKH) is a bilateral, diffuse granulomatous uveitis associated with poliosis, vitiligo, alopecia, and central nervous system and auditory signs. These manifestations are variable and race dependent. This inflammatory syndrome is probably the result of an autoimmune mechanism, influenced by genetic factors, and appears to be directed against melanocytes. On histopathologic examination typical cases show nonnecrotizing diffuse granulomatous panuveitis with initial sparing and late involvement of the choriocapillaris and formation of Dalen-Fuchs' nodules. Fluorescein angiography, lumbar puncture, and echography are useful adjuncts in the diagnosis and management of VKH syndrome. Patients with this syndrome are treated generally with high dose systemic corticosteroids or, when necessary, with cyclosporine or cytotoxic agents. The prognosis of patients with VKH syndrome is fair, with nearly 60% of patients retaining vision of 20/30 or better. The complications of VKH syndrome that lead to visual loss include cataracts in about 25% of patients, glaucoma in 33%, and subretinal neovascular membranes (SRNVMs) in about 10%; the latter, however, are an important cause of late visual loss. These complications usually require medical and/or surgical intervention, including photocoagulation. The major risk factor for the development of cataracts, SRNVMs, and, to some extent, glaucoma, is chronic recurrent intraocular inflammation that may be resistant to corticosteroid therapy. It appears that initial treatment with high dose corticosteroids, combined with prolonged corticosteroid therapy at appropriate dosage, may minimize these complications and may improve visual prognosis.
Article
The Vogt-Koyanagi-Harada syndrome (VKH) is a bilateral, diffuse granulomatous uveitis associated with poliosis, vitiligo, alopecia, and central nervous system and auditory signs. These manifestations are variable and race dependent. This inflammatory syndrome is probably the result of an autoimmune mechanism, influenced by genetic factors, and appears to be directed against melanocytes. On histopathologic examination typical cases show nonnecrotizing diffuse granulomatous panuveitis with initial sparing and late involvement of the choriocapillaris and formation of Dalen-Fuchs' nodules. Fluorescein angiography, lumbar puncture, and echography are useful adjuncts in the diagnosis and management of VKH syndrome. Patients with this syndrome are treated generally with high dose systemic corticosteroids or, when necessary, with cyclosporine or cytotoxic agents. The prognosis of patients with VKH syndrome is fair, with nearly 60% of patients retaining vision of 20/30 or better. The complications of VKH syndrome that lead to visual loss include cataracts in about 25% of patients, glaucoma in 33%, and subretinal neovascular membranes (SRNVMs) in about 10%; the latter, however, are an important cause of late visual loss. These complications usually require medical and/or surgical intervention, including photocoagulation. The major risk factor for the development of cataracts, SRNVMs, and, to some extent, glaucoma, is chronic recurrent intraocular inflammation that may be resistant to corticosteroid therapy. It appears that initial treatment with high dose corticosteroids, combined with prolonged corticosteroid therapy at appropriate dosage, may minimize these complications and may improve visual prognosis.
Article
Vogt-Koyanagi-Harada (VKH) disease is known to have varied manifestations in different ethnic groups. In order to analyze the clinical profile of VKH cases in the Indian population, we studied 87 consecutive cases of VKH disease treated in an uveitis clinic in South India between 1985 and 1996. Retrospective analysis and review of charts of consecutive new VKH cases diagnosed in a referral clinic. VKH disease comprised 2.2% of all uveitis referrals. Extraocular symptoms or signs were seen in 64% of cases at the time of presentation. Most common was meningism (95.9%). However, subsequently all patients developed extraocular manifestations. Panuveitis (92%) was the commonest presentation. Systemic corticosteroid was the usual form of therapy (50.3%) followed by immunosuppressive therapy (39%); surgical treatment was needed in 8% of the cases. Complicated cataract (33%) and glaucoma (24%) were major complications. Final vision was between 6/60 and 6/18 in 88% of the cases and 6/18 and better in 15.4%; there was no improvement in 11% of the cases. VKH disease occurs less frequently in India than in Japan and about as commonly as in the United States. Extraocular signs are far less common than in the Japanese population. Visual prognosis is good in patients presenting within 1 month of onset of symptoms. Immunosuppressive agents and vitreoretinal surgery are needed in advanced cases and in cases reported later. Jpn J Ophthalmol 2000;44:296-301
Article
To present revised criteria for the diagnosis of Vogt-Koyanagi-Harada disease, a chronic, bilateral, granulomatous ocular and multisystem inflammatory condition of unknown cause. Diagnostic criteria and nomenclature were subjects of discussion at the First International Workshop on Vogt-Koyanagi-Harada Disease on October 19-21, 1999, at the University of California, Los Angeles, Conference Center, Lake Arrowhead, California. A committee appointed by the workshop participants was charged with drafting revised criteria for Vogt-Koyanagi-Harada disease, based on discussions held during the conference. This article is the consensus committee report. New criteria, taking into account the multisystem nature of Vogt-Koyanagi-Harada disease, with allowance for the different ocular findings present in the early and late stages of the disease, were formulated and agreed upon by the committee. These criteria are based on additional knowledge and experience of experts in the field and are believed to reflect disease features more fully than previously published criteria. The revised definition of Vogt-Koyanagi-Harada disease, with expanded diagnostic criteria, will facilitate performance of studies involving homogeneous populations of patients, at various stages of disease, that address unanswered questions regarding treatment and disease mechanisms.
Article
The aim of this report was to provide a detailed description of the clinical features of Vogt-Koyanagi-Harada (VKH) disease in Turkish patients. We retrospectively analyzed 45 patients diagnosed with VKH disease at seven referral centers in Turkey. A standard data acquisition form was used for the analysis of demographic and clinical features. The study cohort consisted of 45 patients (32 female, 13 male) who had a mean age at presentation of 31 years. Seven patients (15%) were aged 16 years or younger. Nineteen (42%) patients presented in the acute or subacute stage and 26 (58%) in the chronic stage. According to the revised diagnostic criteria, nine (20%) patients had the complete form of the disease, 23 (51%) had the incomplete form, and 13 (29%) had probable VKH disease. All patients had bilateral ocular involvement. Sunset-glow fundus was observed in 89% of the patients, pigment clumping in 73%, nummular depigmented scars in 71%, cataract in 53%, optic atrophy in 31%, glaucoma in 29%, subretinal fibrosis in 22%, choroidal neovascular membranes in 7%, and phthisis in 4%. All patients who presented at the acute uveitic stage received systemic corticosteroid therapy. Immunosuppressive treatment was employed in 30 (66%) patients, which was initiated at the acute uveitic stage in ten (22%) patients. Final visual acuity was better than 0.5 in 59% of the patients' eyes, between 0.1 and 0.5 in 21%, and less than 0.1 in 20%. VKH disease is rare in Turkey. We conclude that the majority of patients with VKH in Turkey do not have the complete form of the disease. Based on our results, most patients with VKH seem to be late referrals. Ocular complications were common among these patients.