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Year : 2016  |  Volume : 4  |  Issue : 2  |  Page : 97-100

Presumed Vogt-Koyanagi-Harada (VKH) disease in Nepalese population: A rare entity

Department of Ophthalmology, B. P. Koirala Lions Centre for Ophthalmic Studies, Institute of Medicine, Tribhuvan University, Maharajgunj, Kathmandu

Date of Submission26-Nov-2014
Date of Acceptance18-May-2015
Date of Web Publication9-Jun-2016

Correspondence Address:
Ranju Kharel (Sitaula)
Department of Ophthalmology, B. P. Koirala Lions Centre for Ophthalmic Studies, Institute of Medicine, Tribhuvan University, Maharajgunj, Kathmandu

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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2320-3897.183722

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Vogt-Koyanagi-Harada (VKH) disease is a granulomatous inflammatory disorder affecting the eyes, auditory system, meninges, and skin. It typically affects those of more pigmented groups, such as Hispanics, Asians, Native Americans, Middle Easterners, and Asian Indians and is rare among Nepalese population. Hereby, we report a series of eight VKH cases with bilateral ocular involvement, of which six were females. The mean age of the patients was 38.13 ± 11.53 years. The acute uveitic stage was the commonest stage of presentation (4/8). Posterior segment involvement was in the form of disc edema (9/16), serous retinal detachment (5/16), and multifocal choroiditis (5/16). The extraocular manifestations were present in seven cases; neurological manifestations being the commonest.

Keywords: Cataract, glaucoma, sunset glow, uveitis, Vogt-Koyanagi-Harada disease

How to cite this article:
Kharel (Sitaula) R, Shah DN, Chaudhary M. Presumed Vogt-Koyanagi-Harada (VKH) disease in Nepalese population: A rare entity. J Clin Ophthalmol Res 2016;4:97-100

How to cite this URL:
Kharel (Sitaula) R, Shah DN, Chaudhary M. Presumed Vogt-Koyanagi-Harada (VKH) disease in Nepalese population: A rare entity. J Clin Ophthalmol Res [serial online] 2016 [cited 2022 Aug 12];4:97-100. Available from: https://www.jcor.in/text.asp?2016/4/2/97/183722

Vogt-Koyanagi-Harada (VKH) or uveomeningitic syndrome is a bilateral, granulomatous panuveitis associated with nervous, auditory, and integumentary system involvement. It is probably due to cell-mediated autoimmune process driven by T lymphocytes directed against self-antigen associated with the melanocytes in genetically susceptible individuals. [1] This leads to the amplification of inflammatory cascade against the melanocytes of choroid, skin, meninges, and inner ear ultimately resulting in the selective tissue destruction. Revised criteria of First International Workshop on VKH in 1999 defined as bilateral uveitis without a history of trauma or ocular surgery. [2] Meta-analytic study demonstrated a strong association between HLA-DR4/HLA-DRB1*04 and VKH with variable strength of association in different ethnicities. [3] The strength of this association is highest in Eastern Asian and lowest in Indians. [3] No significant association was found between HLA-DRB1*04 and north Indian patients. [4]

This study was conducted to report for the first time from Nepal, a case series of VKH with ocular as well as extraocular manifestations.

  Case Series Top

This study is retrospective review of medical records of VKH patients who presented between 2006 and 2013 at the uveitis clinic of a tertiary eye center of Nepal with 2-8 years follow-up records. Informed consent from patients and approval from Institutional Review Board was obtained. Diagnosis of the presumed VKH was done in all the eight cases based on the revised diagnostic criteria of First International Workshop on VKH. [2] The clinical course of the VKH syndrome with ocular and extraocular manifestations was graded into four stages: prodromal stage (I), acute uveitic stage (II), chronic or convalescent stage (III), and recurrent stage (IV). [5] Based on the number of positive criteria; VKH was categorized as complete, incomplete, or probable VKH disease. [2]

All the eight patients were followed up for 2-8 years by systemic, clinical, ophthalmological, and laboratory examinations. Ocular findings, extraocular manifestations, and response to corticosteroid and immunosuppressant were evaluated.

In our case series, a total of 16 eyes of eight patients of different ethnicity were examined. Baseline characteristics of these eight cases are summarized in [Table 1]. Of them, six were females and two males. Bilateral involvement was present in all the eight cases. The mean age of uveitis onset was 38.13 ± 11.53 years (range, 26-55 years). Four patients presented in acute uveitic stage. The incomplete form of VKH was present in five cases and complete form in three cases. The commonest presenting symptoms were photophobia (six out of 16 eyes) and diminution of vision (four out of 16 eyes). Anterior uveitis was present in 13 eyes; out of which nine were nongranulomatous uveitis and four were granulomatous uveitis with mutton-fat keratic precipitates and iris nodules. Posterior segment finding included disc edema (nine out of 16 eyes), serous retinal detachment (five out of 16 eyes), choroiditis (five out of 16 eyes), and choroidal detachment (two out of 16 eyes). During follow-up, 13 eyes developed sunset-glow fundus [Figure 1] with nummular chorioretinal depigmented scars in the affected eye. The extraocular manifestations were present in seven cases with neurological manifestations being the commonest. Other extraocular features included hearing loss, tinnitus, dizziness, alopecia areata [Figure 2], poliosis, and vitiligo [Figure 3].
Figure 1: The sunset glow fundus with pigmentary changes in chronic VKH case

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Figure 2: The Photograph showing the presence of extraocular features in VKH patient in form of alopecia areata of skull which improved after treatment

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Figure 3: The photograph showing the presence of extraocular features in VKH patient in form of vitiligo of right upper eyelid

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The mean intraocular pressure was 19.4 ± 3 mmHg. Ocular ultrasonography was done in all cases to assess choroidal thickness and retinal edema. The commonest fundus fluorescein angiography finding was multiple spots of pinpoint hyperfluorescence in the early arteriovenous phase with pooling and leakage of the dye in the subretinal space in the late stage of angiogram [Figure 4]. Lumbar puncture was done in two cases of atypical VKH syndrome, which revealed cerebrospinal fluid (CSF) pleocytosis.
Figure 4: The Fundus fluorescein angiography showing multiple spots of pinpoint hyperfluorescence in the early arteriovenous phase

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Table1: Baseline characteristics of the eight VKH cases

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During the acute phase, all eight patients received topical and systemic corticosteroids (oral six cases and intravenous two cases). Recurrent uveitis responded promptly to renewed corticosteroid administration. Immunosuppressant in the form of oral methotrexate had to be added in two patients with persistent chronic uveitis.

Complications observed were complicated cataract (eight out of 16 eyes), secondary glaucoma (four out of 16 eyes), and macular scar (four out of 16 eyes). Four eyes with significant cataract underwent cataract surgery and gained best corrected visual acuity of ≥6/9. Glaucoma filtration surgery was performed in two eyes with medically uncontrolled glaucoma and antiglaucoma agents were discontinued after the filtration surgery.

  Discussion Top

VKH disease is a multisystem disorder. The ocular manifestations are characterized by chronic bilateral, diffuse, granulomatous uveitis, which may lead to blindness. [4] The incidence and prevalence of VKH cases is very uncommon among the Nepalese population and extraocular manifestations are extremely rare.

The mean age at presentation in our series and female preponderance correlates with other studies. [2] Hispanic patients infrequently develop extraocular signs [6] and so was true in our case series. Rubs amen and Gass [7] reported pigment changes, generalized atrophy of the retina, and retinal detachment to be the most common causes of moderate visual loss not explained by cataract which correlates with our study group.

Though a multicenter survey showed high-dose oral corticosteroids as effective as intravenous corticosteroid intervention with similar visual outcomes; [8] in our experience, those patients who did not respond to oral steroid did better after switching to intravenous steroid.

In general, timely establishment of diagnosis and adequate treatment leads to favorable visual prognosis in most of VKH patients. [2] However, age of onset, duration of disease, presence of complications, low visual acuity at onset, severity of intraocular inflammation, late diagnosis, frequent recurrences, and early tapering of steroids may influence the response to treatment and visual outcome.

Posterior subcapsular cataract developed in eight eyes of our patients. Advancing age, long-term corticosteroid therapy, and chronic recurrent anterior segment inflammation seem to be risk factors in the development of posterior subcapsular cataract in upto one-third of all patients. {9}

Human leukocyte antigen (HLA)-DR4/HLA-DRB1*04 has been reported to be a risk factor for VKH disease with various strength of association. [4] Unlike the reports in most populations, in north India, the frequency of HLA-DRB1*04 was 14.6 and 10% in the VKH patients and controls, respectively, and it was not statistically significant. [5]

Study limitations include the lesser number of patients because the rarity of the disease, which itself is the major limitation of this study. We were also unable to perform HLA-DRB1*04 typing and other serological tests due to unavailability of such services in Nepal, so our diagnosis of VKH is only presumed.

  Conclusion Top

VKH is a chronic disease with the potential for significant ocular morbidity. Herein, we have described the various ocular and extraocular characteristics of VKH in Nepalese population in a tertiary care hospital.

  References Top

Matsuda H, Sugiura S. Ultrastructural changes of the melanocyte in Vogt-Koyanagi-Harada syndrome and sympathetic ophthalmia. Jpn J Ophthalmol 1971;15:69-80.  Back to cited text no. 1
Read RW, Holland GN, Rao NA, Tabbara KF, Ohno S, Arellanes-Garcia L, et al. Revised diagnostic criteria for Vogt-Koyanagi-Harada disease: Report of an international committee on nomenclature. Am J Ophthalmol 2001;131:647-52.  Back to cited text no. 2
ShiT, Lv W, Zhang L, Chen J, Chen H. Association of HLA-DR4/HLA-DRB1FNx0104 with Vogt-Koyanagi-Harada disease: A systematic review and meta-analysis. Sci Rep 2014;4:68-87.  Back to cited text no. 3
Gupta A, Kamal S, Gupta V, Bambery P, Kaura B. HLA typing in Vogt-Koyanagi-Harada syndrome in North Indian patients. Ocul Immunol Inflamm 2007;15:89-97.  Back to cited text no. 4
Andreoli CM, Foster CS. Vogt-Koyanagi-Harada disease. Int Ophthalmol Clin 2006:46;111-22.  Back to cited text no. 5
Beniz J, Forster DJ, Lean JS, Smith RE, Rao NA. Variations in clinical features of the Vogt-Koyanagi Harada syndrome. Retina 1991;11:275-80.  Back to cited text no. 6
Rubsamen PE, Gass JD. Vogt-Koyanagi-Harada syndrome. Clinical course, therapy, and long-term visual outcome. Arch Ophthalmol 1991;109:682-7.  Back to cited text no. 7
Read RW. A retrospective multinational survey of current treatment patterns in acute and subacute Vogt-Koyanagi-Harada disease. Association for Research in Vision and Ophthalmology Annual Meeting 2004; April 25-29: VKH Therapy Group. Poster 2664.  Back to cited text no. 8


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

  [Table 1]

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