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Clinical Services at Rush Types of Vasculitis

Behçet’s disease

What is the cause of Behçet’s disease?
What are the symptoms of Behçet’s disease?
How is the diagnosis of Behçet’s disease made?
What is the treatment for Behçet’s disease?

Behçet's disease (Behçet's syndrome), named for Hulusi Behçet, the Turkish dermatologist and scientist who identified this vascular disorder in the 1930s, can cause blood vessels of all sizes throughout the body to become inflamed, although the most typical lesions involve small vessels.

This disease is prevalent in countries of the eastern Mediterranean, the Middle East and the eastern Asian rim. Behçet’s disease occurs primarily in young adults, with the average age of onset between 25 and 30 years.

What is the cause of Behçet’s disease?

The cause of Behçet's disease is unknown, although it may be an autoimmune disorder with genetic and environmental factors.

What are the symptoms of Behçet’s disease?

Behçet's disease symptoms, which vary depending on the individual, may come and go on their own. Common symptoms of this disease include:

  • Canker-type sores in mouth
  • Raised rashes or acne-like sores on skin
  • Ulcers on scrotum or vulva that may leave scars
  • Uveitis, an inflammation of the eye
  • Joint swelling and pain, mostly in the knees
  • Redness, pain and swelling in arms or legs (due to blood clotting)
  • Abdominal pain, diarrhea or intestinal bleeding
  • Headache, fever, disorientation, poor balance or stroke

How is the diagnosis of Behçet’s disease made?

There is no single test to determine the presence of Behçet's disease. The International Study Group (ISG) for Behçet’s disease has proposed the following criteria:

  • Mouth sores. Nearly everyone with Behçet's disease will have mouth sores at some point, so the diagnosis generally requires the recurrence of mouth sores at least three times in 12 months.

In addition, there must be at least two additional signs, such as:

  • Recurring genital sores
  • Eye inflammations
  • Skin rashes or acne-like sores
  • Positive pathergy test. In a pathergy test, a sterile needle is inserted into the skin, and the area is examined two days later. If a small red bump forms under the skin where the needle was inserted, this is an indication that the immune system is overreacting to a minor injury.

In Western countries, large-vessel disease or acute central nervous system infarction (tissue death due to lack of oxygen) may substitute for missing ISG criteria. The multiple signs of Behçet's disease do not necessarily occur at the same time, and in some cases there may be several years between events.

What is the treatment for Behçet’s disease?

No cure exists for Behçet's disease. For mild cases, medications may be prescribed to control temporary flares in pain and inflammation. Skin creams, mouth rinses or eye drops that contain corticosteroids may control inflammation.

Moderate to severe cases of Behçet's disease may warrant the use of corticosteroids (prednisone) to control inflammation, in combination with immunosuppressive medications such as azathioprine (Imuran, Azasan), cyclosporine (Sandimmune) and cyclophosphamide (Cytoxan), or medications that regulate the immune system such as interferon alfa-2b (Intron A).

Other drugs that have been used to treat Behçet's disease include colchicine and methotrexate, infliximab (Remicade), and etanercept (Enbrel).

Medication toxicity remains a major concern in Behçet’s disease. Therefore, it is very important that patients get regular check-ups, be vaccinated, and be constantly monitored for any infection or side effects of therapy. All patients affected by Behçet’s disease should be under the supervision of a skilled health team that is familiar with these conditions, their treatment, and the side effects of immunosuppressive medications.





Contact Name
Rush Vasculitis Clinic
Contact Phone
(312) 563-2800
Contact E-mail
contact_rush@rush.edu



Location
Orthopedic Building
1611 W. Harrison St., Suite 510
Chicago, IL 60612



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