Behcet’s Syndrome

General

Behcet’s syndrome is a disease that involves vasculitis, which is inflammation of the blood vessels. It causes problems in many parts of the body. The most common symptoms are

  • Sores in the mouth
  • Sores on the sex organs
  • Other skin sores
  • Swelling of parts of the eye
  • Pain, swelling and stiffness of the joints

More serious problems can include meningitis, blood clots, inflammation of the digestive system and blindness.

Doctors aren’t sure what causes Behcet’s. It is rare in the United States, but is common in the Middle East and Asia. It mainly affects people in their 20s and 30s. Diagnosing Behcet’s can take a long time, because symptoms may come and go, and it may take months or even years to have all of the symptoms. There is no cure. Treatment focuses on reducing pain and preventing serious problems. Most people can control symptoms with treatment.

Symptoms

Behcet’s disease symptoms vary from person to person. It may disappear and recur on its own. Symptoms may become less severe over time. Your signs and symptoms depend on which parts of your body are affected.

Areas commonly affected by Behcet’s disease include:

  • Mouth. Painful mouth sores that look similar to canker sores are the most common sign of Behcet’s disease. They begin as raised, round lesions in the mouth that quickly turn into painful ulcers. The sores usually heal in one to three weeks, though they do recur.
  • Skin. Skin problems can vary. Some people may develop acne-like sores on their bodies. Others may develop red, raised and tender nodules on their skin, especially on the lower legs.
  • Genitals. People with Behcet’s disease may develop sores on their genitals. The red, open sores commonly occur on the scrotum or the vulva. The sores are usually painful and may leave scars.
  • Eyes. Behcet’s disease may cause inflammation in the eye — a condition called uveitis (u-vee-I-tis). Uveitis causes redness, pain and blurred vision in one or both eyes. In people with Behcet’s disease, the condition and may come and go.
  • Joints. Joint swelling and pain often affect the knees in people with Behcet’s disease. The ankles, elbows or wrists also may be involved. Signs and symptoms may last one to three weeks and go away on their own.
  • Vascular system. Inflammation in blood vessels (veins and arteries) may occur in Behcet’s disease, causing redness, pain, and swelling in the arms or legs when a blood clot results. Inflammation in the large arteries can lead to complications, such as aneurysms and narrowing or blockage of the vessel.
  • Digestive system. Behcet’s disease may cause a variety of signs and symptoms that affect the digestive system, including abdominal pain, diarrhea and bleeding.
  • Brain. Behcet’s disease may cause inflammation in the brain and nervous system that leads to headache, fever, disorientation, poor balance or stroke.

When to see a doctor

Make an appointment with your doctor if you notice any unusual signs and symptoms that might indicate Behcet’s disease. If you’ve been diagnosed with Behcet’s disease, see your doctor if you notice any new signs and symptoms.

Causes

No one knows exactly what causes Behcet’s disease. But it may be an autoimmune disorder, which means the body’s immune system mistakenly attacks some of its own healthy cells. It’s likely that genetic and environmental factors also play a role. Several genes have been found to be associated with the disease. Some researchers believe a virus or bacterium may trigger Behcet’s disease in people who have certain genes that make them susceptible to Behcet’s.

Risk factors

Factors that may increase your risk of Behcet’s include:

  • Age. Behcet’s disease commonly affects men and women in their 20s and 30s, though children and older adults also can develop the condition.
  • Where you live. People from countries in the Middle East and Far East, including Turkey, Iran, Japan and China, are more likely to develop Behcet’s.
  • Sex. While Behcet’s disease occurs in both men and women, the disease is usually more severe in men.
  • Genes. Having certain genes is associated with a higher risk of developing Behcet’s.

Complications

Complications of Behcet’s disease depend on your signs and symptoms. For instance, untreated uveitis can lead to decreased vision or even blindness. People with eye signs and symptoms of Behcet’s disease need to visit an eye doctor (ophthalmologist) regularly because treatment can help prevent this complication.

Diagnosis

No tests can determine definitively whether or not you have Behcet’s disease. Instead, your doctor relies primarily on your signs and symptoms to diagnose Behcet’s disease. He or she may conduct blood tests or other laboratory tests to rule out other conditions.

Criteria have been established for the diagnosis of Behcet’s disease, but these aren’t always essential for the diagnosis of the disease. Your doctor may use other factors for your diagnosis. The classification criteria require:

  • Mouth sores. Because nearly everyone with Behcet’s will have mouth sores at some point, this sign is generally necessary for a diagnosis. The diagnostic criteria require mouth sores that have recurred at least three times in 12 months.

In addition, to receive a diagnosis of Behcet’s disease you must have at least two additional signs, such as:

  • Genital sores. Sores that recur may indicate Behcet’s disease.
  • Eye problems. An ophthalmologist can identify signs of inflammation in your eyes.
  • Skin sores. A variety of rashes or acne-like sores may be caused by Behcet’s disease.
  • Positive pathergy test. In a pathergy test, your doctor inserts a sterile needle into your skin and then examines the area one to two days later. If the pathergy test is positive, a small red bump forms under your skin where the needle was inserted. This indicates your immune system is overreacting to a minor injury.

Treatment

No cure exists for Behcet’s disease. If you have a mild form of the condition, your doctor may offer medications to control temporary flares in pain and inflammation. You may not need to take medication between flares.

If your signs and symptoms are more severe, your doctor may advise medications to control the signs and symptoms of Behcet’s disease throughout your body, in addition to medications for the temporary flares.

Treatments for individual signs and symptoms of Behcet’s disease

Your doctor works to control any signs and symptoms you experience during flares with medications such as:

  • Skin creams, gels and ointments. Topical corticosteroid medicines are applied directly to skin and genital sores in order to reduce inflammation and pain.
  • Mouth rinses. Special mouthwashes that contain corticosteroids and other agents to reduce the pain of mouth sores may ease your discomfort.
  • Eyedrops. Eyedrops containing corticosteroids or other anti-inflammatory medicines can relieve pain and redness in your eyes if inflammation is mild.

Systemic treatments for Behcet’s disease

If topical medications don’t help, your doctor may recommend a drug called colchicine (Colcrys). Arthritis symptoms also may improve with colchicine.

Severe cases of Behcet’s disease require treatments to control damage from the disease between flares. If you have moderate to severe Behcet’s disease, your doctor may prescribe:

  • Corticosteroids to control inflammation. Corticosteroids, such as prednisone, may reduce the inflammation caused by Behcet’s disease. The signs and symptoms of Behcet’s disease tend to recur when corticosteroids are used alone, so doctors often prescribe them with another medication to suppress the activity of your immune system (immunosuppressives).Side effects of corticosteroids include weight gain, persistent heartburn, high blood pressure and bone thinning (osteoporosis).
  • Medications that suppress your immune system. By stopping your immune system from attacking healthy tissues, immunosuppressive drugs reduce the inflammation. Immunosuppressive drugs that may play a role in controlling Behcet’s disease include azathioprine (Imuran, Azasan), cyclosporine (Gengraf, Neoral, Sandimmune) and cyclophosphamide (Cytoxan).Because these medications suppress the actions of your immune system, they may increase your risk of infection. Other possible side effects include liver and kidney problems, low blood counts, and high blood pressure.
  • Medications that alter your immune system’s response. Interferon alfa-2b (Intron A) regulates the activity of your immune system to control inflammation. It may be used alone or with other drugs to help control skin sores, joint pain and eye inflammation in people with Behcet’s disease.Side effects include flu-like signs and symptoms, such as muscle pain and fatigue.

    Medications that block a substance called tumor necrosis factor (TNF) are effective in treating some of the signs and symptoms of Behcet’s, especially for people who have more-severe or resistant symptoms. Examples include infliximab (Remicade) and etanercept (Enbrel).

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