Vogt-Koyanagi-Harada Disease

Abstract

Vogt-Koyanagi-Harada disease (VKH) is a multisystem autoimmune disorder affecting pigmented tissues such as the eye, auditory, integumentary and central nervous systems. Patients are typically women between 20 and 50 years of age with no previous history of penetrating ocular trauma. The clinical course of VKH is divided into four phases: prodromal (mimics a viral infection), uveitic (bilateral diffuse uveitis with papillitis and exudative retinal detachment), convalescent (tissue depigmentation), and chronic recurrent (recurrent uveitis and ocular complications). Clinical, laboratorial, and experimental data corroborate to the autoimmune nature of VKH disease: a CD4+ T-cell-mediated immune response directed against self-antigens found on melanocytes in a genetically susceptible individual. HLA-DRB1*0405 is the main susceptibility allele found in patients with VKH. In accordance with the revised diagnostic criteria, the disease is classified as complete, incomplete, or probable, based on the presence of extraocular findings. The diagnosis of VKH is clinical, and differential diagnosis includes sympathetic ophthalmia, sarcoidosis, primary intraocular B-cell lymphoma, posterior scleritis, and uveal effusion syndrome. Treatment is based on initial high-dose oral corticosteroids with a low tapering during a minimum period of 6 months. Systemic immunomodulatory agents such as cyclosporine may be used in refractory or corticosteroid non-tolerant patients. Visual prognosis is usually good under a prompt diagnosis and adequate treatment.