Update on uveomeningoencephalitides

PURPOSE OF REVIEW: Uveomeningoencephalitides, commonly known as Vogt-Koyanagi-Harada (VKH) disease, are characterized by chronic bilateral granulomatous panuveitis involving the central nervous, auditory and integumentary systems. Visual prognosis is generally favourable, but outcomes in patients with VKH disease may vary. Also, the treatment of choice differs in different parts of the world. This review addresses the literature on the possible pathogenesis, diagnosis and treatment of this disorder. RECENT FINDINGS: Atypical presentations of VKH disease, as well as those associated with interferon-alpha therapy, have been reported. Most reports suggest an association with autoimmunity. The diagnostic criteria were revised by the International Workshop on VKH in 1999, allowing for the presence of different ocular findings in the early and late stages of the disease. New techniques have also been developed to aid in the rapid diagnosis of VKH disease and evaluation of treatment. Different routes of administration of corticosteroid and adjuvant therapy were tried, with positive results. SUMMARY: Although the pathogenesis of VKH disease is uncertain and antigen-specific treatment strategies have not yet been developed, reports increasingly suggest an autoimmune nature for uveomeningoencephalitides. Currently, systemic corticosteroid therapy remains the standard initial treatment. Different routes of administration are used to reduce the frequency of side effects of systemic corticosteroids, and there are various adjuvant therapies. With the aid of modern equipment, early diagnosis and prompt and appropriate treatment, resulting in better visual outcomes, can be anticipated. A large-scale, multinational, prospective study is warranted to determine the optimal initial therapy.