Behcet's Disease

Statistics

• Behçet?s disease is common in the Middle East, Asia, and Japan, but rare in the United States. In Middle Eastern and Asian countries, the disease affects more men than women. In the United States, the opposite is true.

• Behçet?s disease tends to develop in people in their twenties or thirties, but people of all ages can develop it.

What is Behçet?s Disease?

The disease was first described in 1937 by Dr. Helusi Behçet, a professor of dermatology in Istanbul. Behçet?s disease is now recognized as a chronic condition that causes sores or ulcers in the mouth and on the genitals, and inflammation in parts of the eye. In some people, the disease also results in arthritis (swollen, painful, stiff joints) and inflammation of the digestive tract, brain, and spinal cord.

Most people with Behçet?s disease can lead normal lives and control their symptoms with proper medicine, rest, and exercise. Doctors can use many medicines to relieve pain, treat symptoms, and prevent complications. When treatment is effective, flares usually become less frequent after 1 or 2 years. Many patients eventually enter a period of remission. In some people, however, 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 Behçet?s disease.

What Causes Behçet?s Disease?

The exact cause of Behçet?s disease is unknown. Most of the symptoms are caused by inflammation of the blood vessels, particularly veins. Inflammation is the body?s characteristic reaction to injury or disease and is marked by four signs: swelling, redness, heat, and pain. Doctors think that an autoimmune reaction may cause blood vessels to become inflamed, but they do not know what triggers this reaction. In an autoimmune reaction, the immune system mistakenly attacks and harms the body?s own tissues. Under normal conditions, the immune system protects the body from diseases and infections by killing harmful "foreign" substances, such as germs, that enter the body.
Behçet?s disease is not contagious and does not spread from one person to another. Researchers think that two factors are probably important in its development. First, it is believed that abnormalities of the immune system make some people susceptible to the disease. Researchers think that this problem may be inherited; that is, it may be due to one or more specific genes. Second, something in the environment, possibly a bacterium or virus, might trigger or activate the disease in susceptible people. Researchers have found that people who have frequent strep infections (caused by Streptococcus bacteria) are more likely to develop Behçet?s disease.

What are the symptoms?

Behçet?s disease affects each person differently. Some people have only mild symptoms, such as skin sores or ulcers in the mouth or on the genitals. Others have more severe disease, such as meningitis or inflammation of the membranes that cover the brain and spinal cord. Meningitis can cause fever, a stiff neck, and headaches. More severe symptoms usually appear months or years after a person notices the first signs of Behçet?s disease. Symptoms can last for a long time or can come and go in a few weeks. Typically, symptoms appear, disappear, then reappear. The times when a person is having symptoms are called flares. To help the doctor diagnose Behçet?s disease and monitor its course, patients may want to keep a record of the symptoms that occur and when they occur. Because many conditions mimic Behçet?s disease, physicians must observe symptoms to make an accurate diagnosis. The four most common symptoms of Behçet?s disease are mouth sores, genital sores, inflammation of parts of the eye, and arthritis.

How is it diagnosed?

Diagnosing Behçet?s disease is very difficult because no specific test confirms it.

These symptoms are key to diagnosing Behçet?s disease:

Mouth sores at least three times in 12 months
Any two of the following symptoms: recurring genital sores, eye inflammation with loss of vision, skin lesions, or positive pathergy (skin prick test).
Besides finding these signs, the doctor must rule out other conditions with similar symptoms, such as Crohn?s disease and Reiter?s syndrome. The doctor may also recommend that the patient see an eye specialist to identify possible complications related to eye inflammation.

How is it treated?

Medications:

Although there is no cure for Behçet?s disease, people can usually control their symptoms with proper medication, rest, and exercise. Treatment goals are to reduce discomfort and prevent serious complications such as disability from arthritis or blindness. The type of medicine and the length of treatment depend on the person?s symptoms and their severity.

It is likely that a combination of treatments will be needed to relieve specific symptoms. Patients should tell each of their doctors about all of the medicines they are taking so that the doctors can coordinate treatment.

Topical Medicine

Topical medicine is applied directly on the sores to relieve pain and discomfort. For example, doctors prescribe rinses to treat mouth sores. Creams are used to treat skin and genital sores. The medicine usually contains corticosteroids, which reduce inflammation, or an anesthetic, which relieves pain.

Oral Medicine

Doctors also prescribe medicines taken by mouth to reduce inflammation throughout the body, suppress the overactive immune system, and relieve symptoms. Doctors may prescribe one or more of the medicines described below to treat the various symptoms of Behçet?s disease.

Corticosteroids: Prednisone is a corticosteroid prescribed to reduce pain and swelling throughout the body in people with severe joint pain and inflammation, skin sores, eye disease, or central nervous system symptoms. Patients must carefully follow the doctor?s instructions about when to take prednisone and how much to take. It is also important not to stop taking the medicine suddenly because it alters the body?s production of the natural corticosteroid hormones. Long-term use of prednisone can have side effects such as osteoporosis, weight gain, delayed wound healing, persistent heartburn, and elevated blood pressure. However, these side effects are rare when prednisone is taken at low doses for a short time. It is important that patients see their doctor regularly to monitor possible side effects.

Immunosuppressive drugs: Medicines (including corticosteriods) that help control an overactive immune system, such as is the case in people with Behçet?s disease, reduce inflammation throughout the body and can lessen the number of flares. Doctors may use immunosuppressive drugs when a person has eye disease or central nervous system involvement. These medicines are very strong and can have serious side effects. Patients must see their doctor regularly for blood tests to detect and monitor side effects.

Depending on the person?s specific symptoms, doctors may use one or more of the following immunosuppressive drugs:

Azathioprine: Most commonly prescribed for people with organ transplants because it suppresses the immune system, azathioprine is now used to treat uveitis and central nervous system involvement in Behçet?s disease. This medicine can upset the stomach and may reduce the production of new blood cells by the bone marrow.

Chlorambucil: Doctors use chlorambucil to treat uveitis and meningoencephalitis. People taking chlorambucil must see their doctor frequently because it can have serious side effects, such as permanent sterility and cancers of the blood. Patients need regular blood tests to monitor blood counts of white cells and platelets.

Cyclosporine: Like azathioprine, doctors prescribe this medicine for people with organ transplants. When used by patients with Behçet?s disease, cyclosporine reduces uveitis and central nervous system involvement. To reduce the risk of side effects, such as kidney and liver disease, the doctor can adjust the dose. Patients must tell their doctor if they take any other medicines, because some affect the way the body uses cyclosporine.

Colchicine: Commonly used to treat gout, which is a form of arthritis, colchicine reduces inflammation throughout the body. The medicine is sometimes used to treat eye inflammation and skin symptoms in patients with Behçet?s disease. Common side effects of colchicine include nausea, vomiting, and diarrhea. The doctor can decrease the dose to relieve these side effects.

If these medicines do not reduce symptoms, doctors may use other drugs such as cyclophosphamide and methotrexate. Cyclophosphamide is similar to chlorambucil. Methotrexate, which is also used to treat various kinds of cancer as well as rheumatoid arthritis, can relieve Behçet?s symptoms because it suppresses the immune system and reduces inflammation throughout the body.

Rest and Exercise

Although rest is important during flares, doctors usually recommend moderate exercise, such as swimming or walking, when the symptoms have improved or disappeared. Exercise can help people with Behçet?s disease keep their joints strong and flexible.

What research is being done?

Researchers are exploring possible genetic, bacterial, and viral causes of Behçet?s disease, as well as improved drug treatment. Researchers hope to identify genes that increase a person?s chance of developing the disease. Studying these genes and how they work may lead to a new understanding of the disease and possibly new treatments.

Researchers are also investigating factors in the environment, such as a bacterium or virus, that could trigger Behçet?s disease. They are particularly interested in whether Streptococcus, the bacterium that causes strep throat, is associated with the disease. Many people with Behçet?s disease have had several strep infections. In addition, researchers suspect that herpes virus type I, a virus that causes cold sores, may be associated with the disease.

Finally, researchers are identifying other medicines to better treat Behçet?s disease. Thalidomide, for example, appears effective in treating severe mouth sores, but its use is experimental and very limited. Thalidomide is not used in women of childbearing age because it causes severe birth defects.

Some of the related information found on Arthritis Insight:

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For more sites about Behcet's check out our Web Links.

For more information:

American Behcet's Disease Association
The Arthritis Foundation
Johns Hopkins Vasculitis Center: Behcet's Disease
University of Washington: Orthopaedics and Sports Medicine - Behcet's Disease

References:
The National Institute of Arthritis and Musculoskeletal diseases, Questions & Answers about Behcet's