Adamantiades-Behc¸et's Disease

Abstract

Behcet's disease is an immune complex disease characterized by occlusive vasculitis. The disease is most prevalent in the Middle East and the Far East populations but occurs worldwide. The etiology of the disease is multifactorial: infectious agents, immunological mechanisms, and a genetic predisposition, with HLA-B51 as one known risk factor. Behcet's disease can involve blood vessels of nearly all types, including small and large arteries. Therefore, it can affect several organs, including the eyes, mouth, skin, joints, lungs, genitals, and gastrointestinal tract. Diagnosis is based on clinical findings, including recurrent oral ulcerations, evidence of anterior or posterior uveitis, retinal vasculitis, and recurrent genital ulceration. Ocular disease has serious implications and can lead to legal blindness within 4 years if left untreated. Treatment is anti-inflammatory medications such as corticosteroids, cytotoxic agents, cyclosporine, and colchicine. The key to vision preservation is prompt, aggressive therapy. Early treatment suppresses inflammation, reduces the frequency and severity of recurrences, and can halt any involvement of the retina and optic nerve.