What is Behçet's disease?
Behçet's disease or syndrome is a chronic inflammatory condition caused by vasculitis (an inflammation of the blood vessels) that results in damage to blood vessels, particularly veins. The exact cause of Behçet's disease is unknown; it is thought that an autoimmune reaction may cause the blood vessels to become inflamed, but the actual cause is not known.
Who gets Behçet's disease?
Behçet's disease is seen worldwide but most commonly in the Mediterranean basin, Middle East and Far East. The disease can develop at any age but is most common as people reach 20-30 years.
What are the symptoms of Behçet's disease?
Recurrent sores in the mouth and/or genital area, joint pain, and inflammation in the eyes (also called uveitis) characterize Behçet’s disease. Other areas that may be involved are the brain, the nerves, the lungs (rare), the bowels and the kidneys. Behçet's disease affects each person differently.
Various areas of disease involvement are described below:
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Mouth sores: These are usually recurrent and painful and affect almost all individuals with Behçet's disease. The sores look similar to the common canker sore, but the lesions are more numerous, more frequent and more painful. They are often the first symptom that a person notices and may occur long before any other symptoms appear. The mouth ulcers can be found on the lips, tongue, and inside the cheek.
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Genital sores: The sores look similar to the mouth sores and may be painful but they are not as common as the mouth sores. They can be on the scrotum in the male and on the vulva in the female.
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Eye: Behçet’s may cause inflammation in the eye that results in pain, blurry vision, light sensitivity, tearing, or redness of the eye. Behçet’s may even lead to vision loss.
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Skin: Skin problems are a common symptom of Behçet's disease. Skin lesions may resemble acne but are usually more extensive. Other skin lesions include tender nodules over the legs called erythema nodosum. Patients with Behçet's disease may develop a red bump or sore when the skin is scratched or pricked. Doctors refer to this phenomenon as a positive “pathergy test”.
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Joints: Pain in the joints occurs frequently in patients with Behçet's disease. Joints that are most often affected are the ankles, knees, elbows and hips. Inflammation in the joint can cause swelling, redness and tenderness, but usually does not lead to permanent damage.
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Blood clots are common in Behçet's disease and can be in the legs, arms, brain or liver.
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Brain: Behçet's disease can affect the brain. Most commonly the meninges (the membrane that cover the brain) can become inflamed. This may lead to fever, headache, stiff neck, and difficulty coordinating movement. Brain involvement can also include stroke, blockage or rupture of blood vessels in the brain.
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Other organs like the lungs, kidney, and large vessels (such as the aorta) can occasionally be involved.
How is Behçet's disease diagnosed?
There is no single test that can make or confirm the diagnosis of Behçet's disease. The diagnosis is usually made by the presence of compelling signs and symptoms as well as by exclusion of other diseases that may mimic Behçet's disease. An eye exam may be necessary.
How is Behçet's disease treated?
Different medications are used in the treatment of Behçet's disease. The choice of the medications depends on the organ involvement and on the extent of the disease.
The mainstay of treatment is corticosteroids, like prednisone. Colchicine helps with the mouth and genital sores. Other medications called ”immunosuppressants” such as methotrexate, azathioprine and cyclophosphamide can also be used in the treatment course.
New kinds of treatments include a class of medications called “TNF Inhibitors“ (infliximab and etanercept) that have recently been used to treat Behçet's disease.
What is the prognosis?
Behçet's disease is a chronic disease with flares that come and go with varying degrees of intensity. Initially the flares may be more frequent. The disease may go into remission but may strike up again at any time.
Most Behçet's patients will live a normal number of years, but will deal with varying levels of symptoms throughout their lives.
Death occurs in about 4% of Behçet's cases. The most common causes of death are bowel perforation, strokes, and vascular aneurysms (ballooning of blood vessels).