Outcome of Treatment of Uveitis With Biologic Drugs in Resistant Cases Vogt-koyanagi-Harada Disease

Uvea (Latin for grape) is the middle vascular coat of the eyeball. Strictly speaking, uveitis is defined as inflammation of the uvea, but often the inflammatory process involves adjacent intraocular structures e.g. the anterior chamber, the vitreous humor, or the retina. Inflammation in the uvea can be due to infections, masquerades such as B-cell lymphoma, or immune-mediated diseases.

Vogt-Koyanagi-Harada syndrome is a systemic autoimmune disease characterized by bilateral, chronic, diffuse granulomatous panuveitis in addition to neurological, auditory, and integumentary manifestations. These manifestations are variable and race dependent.

This inflammatory syndrome is probably the result of an autoimmune mechanism, influenced by genetic factors, and appears to be directed against melanocytes. On histopathologic examination, typical cases show non-necrotizing diffuse granulomatous panuveitis with initial sparing and late involvement of the choriocapillaris and formation of Dalen-Fuchs' nodules. Fluorescein angiography, lumbar puncture, and echography are useful adjuncts in the diagnosis and management of VKH syndrome. Patients with this syndrome are treated generally with high dose systemic corticosteroids, immunomodulatory agents such as cyclosporine, or more recently biological response modifiers which are used as steroid-sparing agents to minizeminimize side effects of long term steroid use.

Since their discovery in the 1990s, biologic drugs have been used to treat uveitis refractory to traditional immunosuppressants. Many questions on their use though have remained unanswered; particularly when to initiate therapy, which agent and at what dosage to use, and for how long the treatment should continue.

The Biologic Response Modifiers represent the future of therapy in immune-mediated uveitis. Biological anti-inflammatory agents (for example antagonists of tumor necrosis factor alpha-like Infliximab and adalimumab) are also showing very promising results.