What is Behçet's disease?
Behçet's disease or syndrome is a chronic (long term) inflammatory condition caused by vasculitis (an inflammation of the blood vessels) that results in damage to both arteries and veins. The cause of Behçet's disease is unknown.
Who gets Behçet's disease?
Behçet's disease is seen worldwide. It is most common in the Mediterranean basin and Middle East (up to 300 cases/100,000 population) and Far East (about 15 cases/100,000 population). In the US, it is less common (about 7 cases/100,000 population). Men and women are affected with equal frequencies. The disease can develop at any age but most often begins between ages 20-30 years.
What are the symptoms of Behçet's disease?
Recurrent sores in the mouth and/or genital area, skin, joint pain, and inflammation in the eyes are the key symptoms of Behçet’s disease. The brain, nerves, lungs (rare), intestinal tract and kidneys can also be involved.
Where the disease strikes in the body varies from person to person. Some of these sites and key features include:
- Mouth sores occur at some time in all patients. They are usually recurrent and painful and affect almost all patients with Behçet's disease. The sores look like the common canker sore, but are more numerous, frequent and painful. They are often the first symptom that a person notices and may occur long before any other symptoms appear. Mouth ulcers can be found on the lips, tongue, and inside the cheek.
- Genital sores look similar to mouth sores and may be painful, but they are not as common as mouth sores. They can be on the scrotum (the sack enclosing the testes) in the male and on the vulva (the external genital organs) in the female.
- Eye inflammation can result in pain, blurry vision, light sensitivity, tearing, or redness of the eye. Behçet’s may even lead to vision loss. Severe eye disease that can cause blindness is more common in the Middle East and Japan than in the US.
- Skin problems are a common symptom of Behçet's disease. They may look like acne, or have other appearances such as coin-shaped nodules that are tender (called erythema nodosum) or ulcers that may be either superficial or deep and are painful. Patients with Behçet's disease may develop a red bump or sore when the skin is scratched or pricked. Doctors refer to this event as a positive pathergy test.
- Joint pain is common. The most frequently affected sites 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.
- Veins. Inflammation of veins may lead to the development of clots at those sites and the blocking or closing of those vessels. This may affect superficial veins in the skin or deep veins, including the largest in the body (called the vena cava), where the consequences to health are more serious. This is a consequence of inflammation and not a flaw in the clotting system.
- Brain. The brain can be affected, most often the meninges (the brain covering). Inflammation may be associated with fever, headache, stiff neck, and difficulty coordinating movement. Brain involvement can also include stroke, which is due to blockage or rupture of blood vessels in the brain.
- Gastrointestinal (GI) tract features include abdominal pain or blood in the stools, which results from lesions similar to those seen in the mouth and genital area. These lesions can be more dangerous in the GI tract because they are likely to bleed and/or rupture the intestine.
- 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 laboratory test that can make the diagnosis of Behçet's disease. The diagnosis is usually made based on how often oral ulcers come back, plus some of the other features noted above. It is necessary to rule out other illnesses that may also produce mouth sores and mimic Behçet's disease. These illnesses include systemic lupus, Crohn’s disease (an intestinal inflammatory condition) and other forms of vasculitis.
How is Behçet's disease treated?
Different drugs are used to treat Behçet's disease. Drug choice depends on the severity of illness.
Corticosteroids are the main treatment. These drugs suppress inflammation and immune function. One such drug is prednisone. Colchicine may help with mouth and genital sores. There are other drugs that suppress the immune system that are not corticosteroids. Drugs such as methotrexate, azathioprine and cyclophosphamide; and anti-TNF biologics, such as infliximab and etanercept are useful in more severe cases.
What is the prognosis?
Behçet's disease is a chronic disease that may disappear and reappear regardless of treatment. This sometimes makes analysis of the benefits of therapy difficult.
Most Behçet's patients live out a full life, although they will most likely deal with some level of symptoms throughout this time.
Death occurs in about 4% of Behçet's cases. Causes of death are most often intestinal perforation (hole); strokes; and rupture (burst) of enlarged, weakened blood vessels (ie, aneurysms).