Vogt-Koyanagi-Harada Syndrome

PURPOSE: Vogt-Koyanagi-Harada syndrome is a bilateral, chronic, diffuse granulomatous panuveitis frequently associated with neurological, auditory, and integumentary manifestations. It is also one of the most common forms of uveitis among pigmented races including Chinese patients. METHODS: This article reviews the current developments of Vogt-Koyanagi-Harada syndrome, including epidemiology, etiology, clinical features, observational techniques, genetics, treatment, and prognosis. RESULTS: Increasing reports have been published to describe the clinical features of Vogt-Koyanagi-Harada syndrome in various ethnic populations from different parts of the world. In spite of tremendous progress in laboratory and clinical research, the etiology of Vogt-Koyanagi-Harada syndrome is still not completely known. Numerous studies indicate an autoimmune nature for this disease. A recent study has shown that Th17, a new subset of T cell, plays an important role in the initiation and maintenance of this disease. Early and aggressive systemic corticosteroids are still the mainstay of initial therapy for Vogt-Koyanagi-Harada syndrome. However, nonsteroid immunomodulatory therapy, including cyclosporine, chlorambucil, cyclophosphamide, and azathioprine have brought out encouraging results. Improved visual outcomes in patients with Vogt-Koyanagi-Harada syndrome in recent years have been reported when compared with decades ago, presumably due to the more aggressive use of immunosuppressive agents. CONCLUSION: Although the prognosis for VKH syndrome was greatly improved, future prospective, controlled, multi-center studies are needed to determine the optimal treatment regime for this disease. The IL17/23 pathway may provide a novel therapeutic target to control inflammation in VKH syndrome.