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Behcets Disease

(Adamantiales-Behcets Syndrome)

Definition

Behcets disease is a rare, chronic disorder involving inflammation of blood vessels throughout the body. It is marked by recurrent oral and genital ulcers and eye inflammation.

Causes

The cause of Behcets disease is unknown. It is believed to be caused by an autoimmune reaction. This occurs when the body’s immune system attacks its own tissue by mistake. This autoimmune abnormality may be inherited. An environmental trigger, such as a specific virus or bacterium, may activate the disease in people susceptible to it.

Risk Factors

Although the exact cause of Behcets disease is unknown, some groups of people are more likely to develop the condition than others. Behcets disease is more common in people who are 20-39. In the United States, men are more likely than women to develop Behcets. In the Middle East, Asia, and Japan, women are more likely than men to develop Behcets.
Factors that may increase your risk of Behcets include:

Symptoms

Symptoms of Behcets disease can vary from mild to very severe. Symptoms tend to appear, heal, and then recur (referred to as a flare) frequently over months or years. The most common symptoms of the disease are:
Complications of the disease may include:

Diagnosis

Diagnosing Behcets disease is very difficult because:
Behcets disease may be suspected if oral sores appear at least 3 times within a year and at least 2 of the following recurring symptoms appear:
If Behcets disease is suspected, you may have a pathergy skin test. For this test, your skin is pricked with a small needle. If you have Behcets disease, a bump will develop at the site of the skin prick. However, this test is not conclusive. Many people with the disease do not have a reaction to the skin prick.

Treatment

There is no cure for Behcets disease however, spontaneous regression may occur. Treatment is aimed at limiting and preventing complications from symptoms. With proper treatment, the disease can usually be managed fairly well. Treatment includes medications, rest, and exercise.

Medication

Both topical and oral medications are used.
Topical medications include creams, lotions, and mouth rinses that contain corticosteroids (to cut down on inflammation) and/or anesthetics (to decrease pain). These are applied to sores to reduce inflammation and pain.
Oral medications include:
  • Oral corticosteroids—to reduce inflammation and pain
  • Immunosuppressive drugs—to help control the overactive immune system
Many of the drugs used to treat Behcets disease can cause severe side effects. Your doctor must closely monitor you if you are taking these medications.

Rest and Exercise

General guidelines include:
  • Rest when symptoms flare to help speed healing.
  • Engage in moderate exercise when symptoms recede to help keep joints flexible and strong.

Prevention

There are no guidelines for the prevention of Behcets disease because the exact cause is unknown.

RESOURCES

American Behcets Disease Association http://www.behcets.com

National Institute of Arthritis and Musculoskeletal and Skin Diseases http://www.niams.nih.gov

CANADIAN RESOURCES

The Arthritis Society http://www.arthritis.ca

Health Canada http://www.hc-sc.gc.ca

References

Abu El Asrar, ABoooud EB, ALbibhi H, Al-Arfaj A. Long-term safety and efficacy of infliximab therapy in refractory uveitis due to Behcet's disease. Int Ophthalmol. Sep 23, 2006.

Behçet disease. Genetics Home Reference website. Available at: http://ghr.nlm.nih.gov/condition/behcet-disease. Updated July 2010. Accessed June 30, 2015.

Cakir O, Eren N, Ulka R, Nazaroghi H. Bilateral subclavian arterial aneurysm and ruptured abdominal aorta pseudoaneurysm in Behcet's disease. Ann Vasc Surg. 2002,16:516-520

Okada AA. Behcet's disease: general concepts and recent advances. Curr Opin Ophthalmol. 2006;17:551-556.

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