What is Behçet's disease?

Behçet's disease (or syndrome) is a chronic (long-term) inflammatory condition caused by vasculitis (an inflammation, or swelling, of the blood vessels) that results in damage to both arteries and veins. The cause of Behçet's disease is unknown.

Who gets Behçet's disease?

Behçet's disease is seen all over the world. It is most common in the Mediterranean basin and Middle East (up to 300 cases per 100,000 population) and Far East (about 15 cases per 100,000 population). It is less common in the United States (about 7 cases per 100,000 population). Men and women are affected at the same rate. The disease can develop at any age, but most often begins between the ages of 20 and 30.

What are the symptoms of Behçet's disease?

The key symptoms of Behçet's disease are:

  • Sores in the mouth and/or genital area that keep coming back;
  • Skin and joint pain; and,
  • Inflammation in the eyes.

The brain, nerves, lungs (rare), intestinal tract, and kidneys can also be involved.

Where the disease strikes in the body is different for each person. Some of these sites and key features include the following:

  • Mouth sores occur at some time in all patients. They are usually recurrent (keep coming back) and painful and affect almost all patients with Behçet's disease. The sores look like the common canker sore, but are more numerous, frequent, and painful. They are often the first symptom that a person notices, and may occur long before any other symptoms appear. Mouth ulcers can be found on the lips, tongue, and inside the cheek.
  • Genital sores look similar to mouth sores and may be painful, but they are not as common as mouth sores. They can be on the scrotum (the sack enclosing the testes) in the male and on the vulva (the external genital organs) in the female.
  • Eye inflammation can cause pain, blurry vision, light sensitivity, tears, or redness of the eye. Behçet's disease may even lead to vision loss. Severe eye disease that can cause blindness is more common in the Middle East and Japan than in the United States.
  • Skin problems are a common symptom of Behçet's disease. It may look like acne, or like tender, coin-shaped nodules (erythema nodosum), or ulcers that may be either shallow or deep and painful. Patients with Behçet's disease may develop a red bump or sore when the skin is scratched or pricked. Doctors call this a positive pathergy test.
  • Joint pain is common. The most frequently affected sites are the ankles, knees, elbows, and hips. Inflammation in the joint can cause swelling, redness, and tenderness, but usually does not lead to permanent damage.
  • Veins. Inflammation of veins may lead to clots at those sites and the blocking or closing of those veins. This may affect superficial veins (close to the surface) in the skin. It may also affect deep veins, including the largest in the body (the vena cava), which can cause serious health problems. This is a result of inflammation and not a flaw in the clotting system.
  • Brain. The brain can be affected, most often the meninges (the brain covering). Inflammation may be associated with fever, headache, stiff neck, and difficulty coordinating movement. Brain involvement can also include stroke, which is caused by blockage or rupture (bursting) of blood vessels in the brain.
  • Gastrointestinal (GI) tract features include abdominal pain or blood in the stools, which results from lesions similar to those seen in the mouth and genital area. These lesions can be more dangerous in the GI tract because they are likely to bleed and/or rupture the intestine.
  • Other organs, like the lungs or kidneys, and large vessels (such as the aorta) can occasionally be involved.

How is Behçet's disease diagnosed?

There is no single laboratory test that can diagnose Behçet's disease. The diagnosis is usually made based on how often the oral ulcers come back, plus some of the other features noted above.

In order to make a diagnosis, it is necessary to rule out other illnesses that may also produce mouth sores and imitate Behçet's disease, such as systemic lupus, Crohn's disease (an inflammatory condition of the intestines) and other forms of vasculitis. HLA B 51 is a genetic marker that can sometime be present in patients with Behçet's syndrome.

How is Behçet's disease treated?

Different drugs are used to treat Behçet's disease, depending on how severe the illness is:

  • Corticosteroids, such as prednisone, are the main treatment. These drugs suppress inflammation and immune function.
  • Colchicine (Colcrys) may help with mouth and genital sores.
  • Other drugs that suppress the immune system, such as: methotrexate (Trexall, Rasuvo), azathioprine (Imuran, Azasan), and cyclophosphamide (Cytoxan, Neosar); and anti-TNF biologics, such as infliximab (Remicade) and etanercept (Enbrel), are useful in more severe cases.
  • Recently, Apremilast (Otezla), an oral medication (taken by mouth), was found to be effective in controlling mouth ulcers in Behçet's disease.

What is the prognosis (outlook) for patients with Behçet's disease?

Behçet's disease is a chronic disease that may disappear and reappear regardless of treatment. This sometimes makes it difficult to tell how well treatment might be working. Most Behçet's patients live out a full life, although they will probably deal with some level of symptoms throughout this time.

Death occurs in about four percent of Behçet's cases. Causes of death are most often intestinal perforation (hole), strokes, and rupture of enlarged, weakened blood vessels (aneurysms).

References

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