Managing the symptoms of Behçet's disease

Behçet's disease (BD) is a chronic, relapsing, systemic inflammatory vasculitis of unknown aetiology with a myriad of immunological and pathological consequences. Patients with BD are clustered along the ancient silk road, extending from Far-East Asia to Turkey. The disease affects both genders of all ages from infants to the elderly. It is a long-term, cyclical disease and such patients may have symptom-free periods of weeks, months or years that are interrupted by exacerbations of varying intensities lasting a few days, weeks or months. Clinical features include oral aphthae, genital ulcers, ocular inflammation, skin lesions, as well as articular, vascular, neurological, pulmonary, gastrointestinal, renal and genitourinary manifestations. The main histopathological finding is a widespread vasculitis of the arteries and veins of any size or thrombophilia according to the site of involvement. BD may start with just one or two small symptoms but other symptoms may gradually appear over the years. Recurrent ocular inflammation, which occurs in approximately 50% of cases, is the major morbidity that may eventually lead to blindness. The treatment of BD is usually symptomatic and palliative. Therefore, the main objectives are to relieve symptoms associated with mucocutaneous lesions and arthritis, to modify the course of the disease, to control inflammatory eye disease, clinically suppress the inflammation and vasculitis, to prevent recurrences and thus, prevent irreversible damage. The choice of treatment is based on the severity of systemic involvement, clinical presentation and the site affected. The preferred treatment modalities are combined drug therapy and include topical therapies as well as systemic corticosteroids, NSAIDs, colchicine, dapsone and immunosuppressive and cytotoxic agents. Such therapies are tailored to the individual patient depending on clinical manifestations. Thalidomide, tacrolimus, IFN-alpha and anti-TNF monoclonal antibody have recently attracted attention as novel therapeutic approaches.