Behcet's Disease

Behcet's disease is a type of vasculitis characterized by mouth sores, genital sores, inflammation inside of the eye, skin problems, and arthritis (swelling of the joints).The disease usually affects more men than women. The disease appears to be more severe in young, male, and Middle Eastern or Far Eastern patients.

The most striking feature is the presence of painful ulcers (sores). Oral (mouth) ulceration that happens more than three times in one year is considered to be diagnostic for Behcet's disease. Genital ulceration and skin lesions occur in 75% or more of patients. Brain (neurologic) disease occurs in less than one-fifth of patients. Inflammation inside the eye occurs in 25-75 % of patients. Both the small and large blood vessels (the vascular system) can be affected. People who have vascular involvement are at an increased risk of venous thrombosis (a blood clot in a vein). The vessels of the central nervous system (brain and spinal cord) or the heart may also be affected by Behcet's disease.

The underlying cause of Behcet's disease is unknown. There may be environmental or viral factors that make a person’s immune system act differently, due to a genetic predisposition. Investigators are researching these and other possible causes.

There are no specific laboratory tests in Behcet's disease and therefore the diagnosis is made on the basis of the clinical findings.

The available data on treatment of Behcet's disease were systematically reviewed and published in 2000. Ten trials involving 679 patients were included. Some of the classic treatments for Behcet's syndrome appeared to be less effective than previously thought. These included colchicine, cyclophosphamide, and glucocorticoids for eye involvement, azapropazone and colchicine for arthritis, and acyclovir, colchicine, and topical interferon for mouth ulcers. Protective effects were noted with cyclosporine and azathioprine for eye disease and benzathine-penicillin for arthritis.

Several other agents such as Interferon alfa, thalidomide, methotrexate, mycophenolate mofetil, anti-tumor necrosis factor-alpha (TNF) therapy, and infliximab have been used in small studies.

Side effects of treatments for Behcet's disease include suppression of the bone marrow and the immune system leading to a greater chance of getting infections.
Corticosteroids are associated with increased risk of diabetes and bone mineral loss depending on the total period of treatment as well as the specific characteristics of the patient (gender, age). The use of Cyclosporine may cause hypertension (high blood pressure) and glucose intolerance. Cyclosporine is also considered to be damaging to the kidney, so close monitoring of the kidneys is needed.

Prognosis: Most people with Behcet's disease can lead productive lives and control symptoms with proper medication, rest, and exercise. When treatment is effective, flares usually become less frequent. Many patients eventually enter a period of remission (a disappearance of symptoms). Sometimes, treatment does not relieve symptoms, and gradually more serious symptoms such as eye disease may occur. Serious symptoms may appear months or years after the first signs of Behcet's disease occur.