Vogt–Koyanagi–Harada disease in Hispanic patients
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- Sukavatcharin, S., Tsai, J.H. & Rao, N.A. Int Ophthalmol (2007) 27: 143. doi:10.1007/s10792-006-9017-6
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To describe the clinical features of Vogt–Koyanagi–Harada disease (VKH) in Hispanic patients.
Retrospective review of the records of 48 Hispanic patients diagnosed with VKH. The patients were divided into two groups: patients in the early phase of VKH (n = 11) were those who presented within 1 month after the onset of symptoms; patients in the late or chronic VKH phase (n = 37) were those who presented 6 months after onset of symptoms. Demographic data, clinical features, complications and initial and final visual acuity for each patient were recorded.
All 11 patients in early phase VKH presented with bilateral uveitis (100%). Meningismus was noted in six cases and auditory disturbances in three. Ocular findings for these 11 patients included exudative retinal detachment in ten patients (91%) and marked optic disc edema in one patient. In the late phase VKH, ocular findings included sunset glow fundus in 26 patients (70%), peripheral nummular scars in 27 (73%), and retinal pigment epithelium hyperplasia in seven (19%). Extraocular manifestations noted in this group of patients included vitiligo in four, poliosis in six, and alopecia in five; auditory disturbances were found in four patients. The visual acuity improved in 60–70% of the patients after treatment with corticosteroids alone or in combination with immunosuppressive agents.
Hispanic patients with VKH often present without extraocular changes during early phase of the disease. However, once the disease evolves into the chronic phase, integumentary system involvement may become apparent in some patients.
KeywordsCorticosteroidEarly phase VKHHispanicLate phase VKHVogt–Koyanagi–Harada disease
Vogt–Koyanagi–Harada disease (VKH) is found worldwide and primarily affects darkly pigmented races, including Hispanic, Asian, Native American and Asian Indians. The disease is relatively uncommon in whites and African–Americans [1, 2]. It is associated with HLA-DRB1*0405 in Asian populations and with HLA-DRB1*04 subtypes in Hispanic patients [3, 4]. Vogt–Koyanagi–Harada disease appears to be more prevalent in Japan, where it accounts for between 6.8% and 9.2% of all uveitis referrals . In the United States of America, VKH accounts for between 1% and 4% of all uveitis clinic referrals [6, 7]. A series reported by the National Institutes of Health (NIH), Bethesda, Maryland, showed that 50% of VKH patients were white, 35% were African-American, and 13% were Hispanic . In northern California, VKH is seen in Asians (41%), whites (29%), Hispanics (16%) and African–Americans (14%). In contrast, the majority of patients with VKH in southern California are Hispanic (78%), with the remainder coming from the Asian (10%), African–American (6%) and white (3%) populations .
Vogt–Koyanagi–Harada disease is known to have varied manifestations in different ethnic groups. For instance, in Japanese patients, dysacusia occurs in 70–80%, poliosis or alopecia in 60%, and vitiligo in 25%; however, dysacusia was noted in 8%, poliosis in 25%, and alopecia in 8% of patients in a series of VKH cases reported from San Francisco [6, 10, 11]. Since the clinical features of VKH vary depending upon the stage of the disease, patients rarely have all of these features during the initial presentation. The difference in proportions of racial groups may thus reflect discrepancies in the populations from which the patients are derived. The purpose of this study is to clarify the clinical features and visual outcomes in Hispanic patients with early and late phase VKH.
Patients and methods
A retrospective review was conducted on the charts of 48 patients diagnosed with VKH who were identified as Hispanic. The patients were divided into two groups. The first group (early phase VKH, n = 11) presented within 1 month after the onset of symptoms, which included headache, decreased vision, photophobia or floaters. The second group (n = 37) included those patients who presented 6 months after the onset of symptoms (chronic or late phase VKH).
Charts were reviewed for demographic data, such as age and gender, and for clinical features, such as ocular and extraocular manifestations in early and late phase VKH. The results of laboratory investigations, such as lumbar puncture, ultrasonography and fluorescein angiography, were also noted. Ocular complications that were noted included cataract, glaucoma, subretinal neovascularization or fibrosis, cystoid macular edema and epiretinal membrane. Visual acuity was documented at the initial and final visits.
Clinical manifestations of VKH in Hispanic patients during early and late phase
Early phase (11 patients) (%)
Late phase (37 patients) (%)
Exudative retinal detachment
Optic disc swelling
Sunset glow fundus
Retinal pigment epithelium hyperplasia
Lumbar puncture was performed on two patients. Laboratory analysis of the cerebrospinal fluid showed pleocytosis in both patients. Fluorescein angiography performed on seven patients (64%) revealed multiple pinpoint hyperfluorescent leakage at the level of the retinal pigment epithelium and pooling of dye in the subretinal space. Ultrasonography confirmed diffuse bilateral choroidal thickening and overlying serous retinal detachments in three patients (27%).
On initial presentation all 37 patients in the chronic phase VKH group showed inflammatory cell infiltration of the vitreous and anterior chamber. Twenty-six patients (70%) had sunset glow fundus. Peripheral nummular scars were noted in 27 patients (73%), and retinal pigment epithelium atrophy and hyperplasia were found in seven (19%). Sugiura’s sign was seen in only one patient. One or two integumentary changes were noted in seven patients (19%); vitiligo was seen in four (11%), poliosis in six (16%) and alopecia in five (14%). Auditory disturbances were noted in four patients (11%).
All 48 patients were initially treated with orally administered corticosteroids or corticosteroids combined with immunosuppressive/cytotoxic agents. The starting dose of orally given corticosteroid for patients presenting in the acute phase of VKH was 1–1.5 mg/kg per day. Marked resolution of exudative retinal detachment was seen within 3–6 weeks in nine patients (82%). The remaining two patients (18%) showed resolution by 8–12 weeks. Those patients who presented during the chronic phase of VKH were treated with corticosteroid 1 mg/kg alone (23 patients) or in combination with immunosuppressive agents (14 patients). The immunosuppressive agents used included weekly doses of methotrexate 15–22.5 mg (two patients), azathioprine 100–150 mg (six patients), cyclosporin 2.5–5 mg/kg (five patients) and mycophenolate mofetil 1,000 mg b.i.d. (one patient).
Ocular complications in 24 patients with VKH
Number of patients and percentage
Subretinal neovascularization or subretinal fibrosis
Cystoid macular edema
Summary of initial and final visual acuity in Hispanic patients presenting with early and late phase VKH (VA visual acuity)
Early phase (22 eyes)
Late phase (74 eyes)
Number of eyes and percentage
Number of eyes and percentage
20/20 to 20/50
20/50 to 20/200
20/200 or less
The integumentary signs did not develop in Hispanic patients with VKH until the chronic phase of the disease. This is similar to the reported findings for Japanese and other ethnic groups. Although skin involvement was seen in about 19% of Hispanic patients with VKH, by comparison, such manifestations are still less common than in other series that reported an incidence of integumentary disorders ranging from 25% to 60% [2, 6, 7, 11]. This variability in clinical presentation thus underscores the necessity for a broader set of diagnostic criteria, such as that seen in the revised diagnostic criteria developed by the International Uveitis Study Group . Moreover, newer investigation such as indocyanine green angiography may be helpful in demonstrating choroidal inflammatory changes in patients with atypical clinical presentation .
The ocular complications of VKH appear to be common in those patients who are not properly treated during the early phase. There are many possible causes of decreased vision in patients with VKH. During episodes of initial or recurrent uveitis, vision may be limited by the inflammation itself, by serous elevation of the neurosensory retina, or, rarely, by cystoid macular edema . Once the inflammation has been controlled, complications such as subretinal fibrosis may develop as long-term complications of VKH . Other issues, such as the development of cataract , glaucomatous optic nerve damage , widespread alterations in the retinal pigment epithelium (RPE), and loss of choroidal melanocytes may also contribute to visual loss. Thus, it is important to establish an early and accurate diagnosis of VKH [19, 20]. The complication rate seen in the Hispanic patients is similar to that seen in non-Hispanic patients , suggesting that the response to systemic therapy is similar in Hispanic and non-Hispanic individuals.
Treatment with systemic corticosteroids is known to alter the clinical features and course of VKH [19, 22]. Treatment can prevent the development of complications and may reduce the frequency of depigmentation in the eyes and other melanocyte-containing systemic organs [19, 20, 23]. Ohno et al.  suggested that early administration of corticosteroids may help delay the appearance of extraocular manifestations, which may preclude the development of the full spectrum of disease manifestations.
In conclusion, the current study shows that extraocular manifestations of VKH may differ in Hispanic populations compared with other ethnic groups. In our series of Hispanic patients the extraocular manifestations, including neurological and dermatologic findings, were less common. However, intraocular changes were similar to those reported for other ethnic groups. The overall prognosis for adequately managed cases is fair, with nearly 60–70% of patients having a final visual acuity of 20/50 or better.
Supported in part by NIH core grant EY03040 and by a grant from Research to Prevent Blindness, Inc., New York, NY., USA