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R.P. Werner, D.G. Chamberlain, T.H. Mader; Familial Vogt-Koyanagi-Harada Syndrome in Alaskan Natives . Invest. Ophthalmol. Vis. Sci. 2003;44(13):2385.
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Purpose: Vogt-Koyanagi-Harada Syndrome has predilection for certain racial groups but rare familial transmission. In the U.S. most patients share a Hispanic or American Indian ancestry. This suggests genetic factors may predominate in the susceptibility to the development of this disease. Alaska Natives have a high incidence of V-K-H similar to their Pacific Rim neighbors. We report the first examples of Alaska Natives in familial groups with this disease. Methods: A retrospective review of Alaska Natives presenting at the Alaska Native Medical Center with V-K-H over the last 30 years. Two family groups were identified with members having this disease. An analysis of the cases was done to identify severity, presenting signs, treatment, complications and outcomes. Results: A total of five patients from two families were identified. All came from small rural communities having less then 200 inhabitants. In one case two sisters out of five siblings presented with classic signs 25 years apart. The first at age 15 the second at age 48. The second family had a mother/daughter/cousin combination. All patients were female. Eyes uniformly presented with exuditive retinal detachments or papillitis with panuvietis. Common signs of central nervous system involvement were tinnitus, hearing loss and headache. Dermatological involvement with poliosis and alopecia occurred in only one patient. Complications included Cataract, and NVM. The course of the disease, which responded in all cases to standard treatment with long term high dose steroids, was prolonged with 3 out of 5 maintaining chronic recurrent disease. Conclusion: These cases are the first reported families of Alaska Natives with V-K-H. Shared genetic similarities along with commonly experienced environmental effects are probably responsible for the vulnerability of these families to the syndrome. The variation in the severity of the signs and outcomes is characteristic for this syndrome.
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