Vogt-Koyanagi-Harada Disease: Current Diagnosis and Management

Abstract

Vogt-Koyanagi-Harada (VKH) disease is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear, meninges, skin, and hair. This disease is mainly a T1 lymphocyte-mediated aggression to melanocytes. The availability of new investigational methods has improved our knowledge of the pathogenesis, clinical approach, diagnosis, and management of VKH disease. The disease has an acute onset of bilateral blurred vision with hyperemia in the absence of prior ocular trauma or any previous intraocular surgery. The chronic phase comprises of ocular and integumentary system pigmentary changes. Ocular findings may be accompanied by meningismus, hearing impairment, and skin lesions in a variable proportion of patients. Prompt diagnosis with early, aggressive, and long-term treatment of high-dose corticosteroids ensures good visual outcomes. The aim of this chapter is to present the clinicopathology, classification, recent imaging, investigations, and management of VKH disease.