Vogt-Koyanagi-Harada Disease

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 females between 20 and 50 years old 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, laboratory, and experimental data corroborate 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 primary susceptibility allele found in patients with VKH. According to 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 immunosuppressants, such as cyclosporine, azathioprine, mycophenolate mofetil, and adalimumab, may be used in refractory or corticosteroid non-tolerant patients. Visual prognosis is usually good under a prompt diagnosis and adequate treatment.