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Behcets Disease

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. Factors that may increase your risk of Behcets include:

  • Location: the Middle East, Asia, and Japan
  • Family history of Behcets disease
  • A gene marker called the HLA-B51/B5 allele
  • Sex:
    • In the United States, men are more likely than women to develop this condition.
    • In the Middle East, Asia, and Japan, women are more likely than men to develop Behcets.
  • Age: 20's and 30's

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:

  • Oral sores
    • Affect almost all people with the disease
    • Usually are the first to appear
    • Usually last for 10-14 days
    • Can be painful
    • Can cause scarring
  • Ulcerated Gums
    Periodontal Disease
    Copyright © Nucleus Medical Media, Inc.
  • Genital sores
    • In men: appear on the penis and/or scrotum
    • In women: appear on the vulva or within the vagina
    • Can be painful
    • Can cause scarring
  • Uveitis—inflammation of the middle part of the eye, including the iris
    • Tends to cause eye redness, blurred vision, sensitivity to light, and watering eyes
    • If not treated, can cause partial vision loss or blindness
  • Arthritis —inflammation of joints
    • Tends to be painful
    • Usually does not cause permanent joint damage
  • Skin problems, such as sores
    • May appear as red, raised sores or bumps, or may be flat (flush with the skin)
    • Usually appear on the legs and upper torso

Other symptoms of the disease may include:

  • Severe fatigue during a flare
  • Blood clots
    • Caused by thrombophlebitis (inflammation of veins), usually in the legs
    • Can cause severe complications if not treated quickly
  • Aneurysms , or severely dilated blood vessels
    • May rupture and cause severe consequences
  • Heart problems, such as:
    • Abnormal heart rhythms
    • Missed heartbeats
    • Early heartbeats
    • Inflammation of the heart muscle ( myocarditis )
  • Central nervous system problems
    • For example, meningoencephalitis, which is inflammation of the brain and membrane that lines the brain
    • May result in seizures , confusion, strokes , memory problems, headaches
  • Stroke from a Cerebral Aneurysm
    Cerebral Aneurysm
    Copyright © Nucleus Medical Media, Inc.
  • Digestive problems
    • Causes ulcers and inflammation of the digestive tract (rarely)

Diagnosis

Diagnosing Behcets disease is very difficult because:

  • Symptoms are similar to those of many other diseases.
  • Symptoms often appear very slowly, over months or years.
  • There is no specific test to confirm the disease.

A doctor may suspect Behcets disease if oral sores appear at least three times within a year and at least two of the following recurring symptoms appear:

  • Eye inflammation
  • Genital sores
  • Skin sores

If Behcets disease is suspected, your doctor will do tests to rule out other diseases with similar symptoms. In addition, 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. These may include:
    • Interferon
    • Azathioprine
    • Chlorambucil
    • Cyclosporine
    • Colchicine
    • Methotrexate
    • Dapsone
    • Infliximab
    • Thalidomide

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.

Revision Information

  • American Behcets Disease Association

    http://www.behcets.com

  • National Institute of Arthritis and Musculoskeletal and Skin Diseases

    http://www.niams.nih.gov

  • The Arthritis Society

    http://www.arthritis.ca

  • Health Canada

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

  • 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.

  • 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

  • The Medical Letter. 1996;38:15.

  • The Merck Manual of Diagnosis and Therapy. Simon & Schuster; 2001.

  • National Organization of Rare Disorders, Inc. website. Available at: http://www.rarediseases.org/ .

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

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