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Questions and Answers About Behcets Disease: NIAMS

Article title: Questions and Answers About Behcets Disease: NIAMS

Conditions: Behçet’s Disease

Source: NIAMS


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Publication Date: January 1999

Questions and Answers About Behçet’s Disease

This fact sheet contains general information about Behçet’s (buh.SETZ) disease. It describes what Behçet’s disease is and how it may develop. It also explains how Behçet’s disease is diagnosed and treated. At the end is a list of key words to help you understand the terms used in this fact sheet. If you have further questions after reading this fact sheet, you may wish to discuss them with your doctor.

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.

Who Gets Behçet’s Disease?

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 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 of Behçet’s Disease?

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.

  • Mouth sores—Mouth sores (known as oral aphthosis and aphthous stomatitis) affect almost all patients with Behçet’s disease. They are often the first symptom that a person notices and may occur long before any other symptoms appear. The sores usually have a red border and several may appear at the same time. They can be painful and make eating difficult. Mouth sores go away in 10 to 14 days but often come back. Small sores usually heal without scarring, but larger ones may scar.
  • Genital sores—Affecting more that half of all people with Behçet’s disease, most genital sores appear on the scrotum in men and vulva in women. The sores look similar to mouth sores and may be painful. After several outbreaks, they may cause scarring.
  • Uveitis (yoo.vee.EYE.tis)—Inflammation of the middle part of the eye (the uvea), including the iris, occurs in more than half of all people with Behçet’s disease. This symptom is more common among men than women and typically begins within 2 years of the first symptoms. Eye inflammation can cause blurred vision and, rarely, pain and redness. Because partial loss of vision or blindness can result if the eye frequently becomes inflamed, patients should report these symptoms to their doctor immediately.
  • Arthritis—Inflammation of the joints occurs in more than half of all patients with Behçet’s disease. Arthritis causes pain, swelling, and stiffness in the joints, especially the knees, ankles, wrists, and elbows. Arthritis that results from Behçet’s disease usually lasts a few weeks and does not cause permanent damage to the joints.

In addition to mouth and genital sores, eye inflammation, and arthritis, Behçet’s disease may cause other skin problems, blood clots, and inflammation in the central nervous system and digestive organs.

Skin Problems

Behçet’s disease causes various skin sores that look like red bumps on a black-and-blue mark. The sores are red, raised, and typically appear on the legs and upper torso. In some people, sores or lesions may appear when the skin is scratched or pricked. When doctors suspect that a person may have Behçet’s disease, they may perform a test called pathergy in which they prick the skin with a small needle: 1 to 2 days after the test, people with Behçet’s disease may develop a bump where the doctor pricked the skin. Doctors disagree about the usefulness of a pathergy test because Behçet’s patients in the United States rarely have a skin reaction. However, more than half of the patients in Middle Eastern countries and Japan do have a reaction.

Blood Clots

About 10 percent of patients with Behçet’s disease have blood clots resulting from inflammation in the veins (thrombophlebitis), usually in the legs. Symptoms include pain and tenderness in the affected area, which may also be swollen and warm. Because thrombophlebitis can have severe complications, people should report symptoms to their doctor immediately. A few patients may experience artery problems such as aneurysms (a stretching or expanding of a weakened blood vessel).

Central Nervous System

Behçet’s disease affects the central nervous system in about 10 percent of all patients with the disease. The central nervous system includes the brain and spinal cord and helps the body to coordinate movements and process information. Behçet’s disease can cause meningoencephalitis— inflammation of the brain and the thin membrane that covers and protects it. People with meningoencephalitis may have fever, headache, stiff neck, and difficulty coordinating movement, and should report any of these symptoms to their doctor immediately. If this condition is left untreated, a stroke can result.

Digestive Tract

Only rarely does Behçet’s disease cause inflammation and ulceration (sores) in the digestive tract and lead to stomach pain, diarrhea, constipation, and vomiting. Because these symptoms are very similar to symptoms of other diseases of the digestive tract, such as a peptic ulcer, ulcerative colitis, and especially Crohn’s disease, careful evaluation is essential.

How Is Behçet’s Disease Diagnosed?

Diagnosing Behçet’s disease is very difficult because no specific test confirms it. Less than half of the patients initially thought to have Behçet’s disease actually have it. The doctor must examine a patient with symptoms and rule out other conditions with similar symptoms. Because it may take several months or even years for all the common symptoms to appear, the diagnosis may not be made for a long time. A patient may even visit several different kinds of doctors before the diagnosis is made.

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.

What Kind of Doctor Treats a Patient With Behçet’s Disease?

Because the disease affects different parts of the body, a patient will probably see several different doctors. It may be helpful to both the doctors and the patient for one doctor to manage the complete treatment plan. This doctor can coordinate treatment and monitor any side effects from the various medications the patient takes.

A rheumatologist (a doctor specializing in arthritis) often manages the patient’s overall treatment and treats joint disease. The following specialists treat other symptoms that affect different body systems:

  • Gynecologist—treats enital sores in women.
  • Urologist—treats genital sores in men.
  • Dermatologist—treats genital sores in men, and skin and mucous membrane problems.
  • Ophthalmologist—treats eye inflammation.
  • Gastroenterologist—treats digestive tract symptoms.
  • Neurologist—treats central nervous system symptoms.

How Is Behçet’s Disease Treated?

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 Is the Prognosis for a Person With Behçet's Disease?

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 Are Researchers Trying To Learn About Behçet's Disease?

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.

Where Can People Get More Information About Behçet's Disease?

  • Arthritis Foundation
    1330 West Peachtree Street
    Atlanta, GA 30309
    404/872-7100
    800/872-7800, or call your local chapter (listed in the telephone directory)
    World Wide Web address: http://www.arthritis.org/
  • American Behçet’s Disease Association
    P. O. Box 6663
    Minneapolis, MN 55406-0663
    800/7BEHCET (800/723-4238)
  • National Arthritis and Musculoskeletal and
    Skin Diseases Information Clearinghouse (NAMSIC)

    National Institutes of Health
    1AMS Circle
    Bethesda, MD 20892-3675
    301/495-4484
    Fax: 301/718-6366
    TTY: 301/565-2966
    World Wide Web address: http://www.niams.nih.gov/index.htm

Acknowledgments

The NIAMS gratefully acknowledges the assistance of J. Desmond O. Duffy, M.D., of the Mayo Clinic, and Joseph L. Jorizzo, M.D., Wake Forest University Medical Center, in the preparation and review of this fact sheet.

Keywords

Anesthetic: Pain relief medicine that dulls feeling in the sore area. Doctors sometimes use an anesthetic to relieve pain caused by mouth and genital sores in people with Behçet's disease.

Aneurysm: A stretching or expanding of a weakened blood vessel.

Antibody: A special protein produced by the body·s immune system that recognizes and helps fight infectious agents and other foreign substances that invade the body.

Aphthosis: Ulcers in the mouth or on the genitals. Oral or mouth aphthosis, also called aphthous stomatitis, is the most common symptom of Behçet's disease.

Arthritis: Literally means joint inflammation. It is a general term for more than 100 conditions known as rheumatic diseases. Arthritis causes joint swelling, pain, and stiffness. Some people with Behçet's disease develop a form of arthritis that goes away after a few weeks or months and causes no long-term damage to the joints.

Autoimmune disease: A disease in which the body's immune system destroys or attacks its own healthy tissues.

Blood vessels: Arteries, veins, and capillaries that carry blood through the body.

Central nervous system: Includes the brain and spinal cord. Its function is to process information and coordinate thinking and movement. In some people with Behçet's disease, blood vessels in the central nervous system become inflamed, causing headaches, stiff neck, and clumsiness.

Corticosteroids: Strong anti-inflammatory hormones that are made naturally in the body or synthetically for use as medicine. They are also called glucocorticoids. The most commonly prescribed drug of this type is prednisone.

Crohn's disease: Inflammation of the small intestine or colon that causes diarrhea, cramps, and weight loss.

Digestive tract: The body system that breaks down food. The digestive tract includes the stomach, intestines, pancreas, and liver.

Flare: A period of time when disease symptoms reappear or become worse.

Immune system: A complex network of specialized cells and organs that work together to defend the body against attacks by ·foreign· invaders such as bacteria and viruses. In some rheumatic conditions, it appears that the immune system does not function properly and may even work against the body.

Immunosuppressive drugs: Medicines that decrease the immune response and can therefore relieve some symptoms of Behçet's disease.

Inflammation: A reaction of tissues to injury or disease, marked by four signs: swelling, redness, heat, and pain.

Meningitis: Inflammation of the membranes of the brain or spinal cord.

Meningoencephalitis: Inflammation of the brain and its membranes. This condition sometimes occurs in patients with Behçet's disease.

Pathergy: A test that detects skin sensitivity and is sometimes used to help diagnose Behçet·s disease. The skin is pricked with a small needle and, in some people, a bump appears after 1 or 2 days. This reaction is called positive pathergy.

Peptic ulcer: A sore on the wall of the stomach.

Reiter's syndrome: A form of arthritis that can develop after an intestinal or urinary tract infection. The disease causes pain and swelling around the joints and in the spine. People with the disease may also experience swelling of the eye and the reproductive and urinary tracts.

Streptococcus: A bacterium that causes infections such as strep throat. Doctors think that it may also trigger Behçet's disease in some people.

Thrombophlebitis: Inflammation of a vein and formation of a blood clot in the vein.

Topical treatment: Medicine, such as a cream or rinse, that is put directly on the affected body part.

Ulcerative colitis: Inflammation of the colon. Symptoms include stomach pain and diarrhea.

Uveitis: Inflammation of the inner eye that includes the iris, the tissue that holds the lens of the eye, and a network of blood vessels surrounding the eyeball called the choroid plexus.

The National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS), a part of the National Institutes of Health (NIH), leads the Federal medical research effort in arthritis and musculoskeletal and skin diseases. The NIAMS supports research and research training throughout the United States, as well as on the NIH campus in Bethesda, MD, and disseminates health and research information. The National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse (NAMSIC) is a public service sponsored by the NIAMS that provides health information and information sources. Additional information can be found on the NIAMS Web site at http://www.niams.nih.gov/index.htm


 

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