Behçet disease

What causes Behçet disease?

The exact cause of Behçet disease is unknown. Most symptoms of the disease are caused by inflammation of the blood vessels (vasculitis). Inflammation is a characteristic reaction of the body to injury or disease and is marked by four signs: swelling, redness, heat, and pain. Doctors think that an autoimmune reaction may cause the blood vessels to become inflamed, but they do not know what triggers this reaction. Under normal conditions, the immune system protects the body from diseases and infections by killing harmful "foreign" substances, such as germs, that enter the body. In an autoimmune reaction, the immune system mistakenly attacks and harms the body's own tissues. Behçet disease is not contagious; it does not spread from one person to another.

Researchers think that two factors are important for a person to develop Behçet disease:

  • First, it is believed that abnormalities of the immune system make some people susceptible to the disease. Scientists think that this susceptibility may be inherited; that is, it may be due to one or more specific genes. People with the disease are more likaly to have certain “human leukocyte antigens” (HLAs) in the blood, especially HLA-B51, than the general population. Other genetic markers and their role in the development of Behçet’s disease are being studied.
  • Second, something in the environment, possibly a bacterium (such as Helicobacter pylori) or virus (such as Herpes simplex virus, and parvovirus B19) or exposition to certain substances (such as heavy metals), might trigger or activate the disease in susceptible people.

Last updated on 05-01-20

What is the prognosis for individuals with Behçet disease?

Most people with Behçet disease can lead productive lives and control symptoms with proper medicine, rest, and exercise. Doctors can use many medicines to relieve pain, treat symptoms, and prevent complications. When treatment is effective, flares usually become less frequent. Many patients eventually enter a period of remission (a disappearance of symptoms). In some people, treatment does not relieve symptoms, and gradually more serious symptoms such as eye disease may occur. Serious symptoms may appear months or years after the first signs of Behçet disease.

Last updated on 05-01-20

How might Behçet disease be treated?

Although there is no cure for Behçet disease, people can usually control symptoms with proper medication, rest, exercise, and a healthy lifestyle. The goal of treatment is to reduce discomfort and prevent serious complications such as disability from arthritis or blindness. The type of medicine and the length of treatment depend on the person's symptoms and their severity. It is likely that a combination of treatments will be needed to relieve specific symptoms. Patients should tell each of their doctors about all of the medicines they are taking so that the doctors can coordinate treatment.

Topical medicine is applied directly on the sores to relieve pain and discomfort. For example, doctors prescribe rinses, gels, or ointments. Creams are used to treat skin and genital sores. The medicine usually contains corticosteroids (which reduce inflammation), other anti-inflammatory drugs, or an anesthetic, which relieves pain.

Doctors also prescribe medicines taken by mouth to reduce inflammation throughout the body, suppress the overactive immune system, and relieve symptoms. Doctors may prescribe one or more of the medicines listed below to treat the various symptoms of Behçet disease.

Interferon-alfa, azathioprine, and TNF-α blockers may be tried in rare cases of patients with resistant, prolonged, and disabling attacks.

The European League Against Rheumatism (EULAR) has recommendations for the management of Behçet disease.

For ocular disease, azathioprine is the first medication that should be used. For severe eye disease (such as drop in visual acuity, retinal vasculitis, or macular involvement), either cyclosporine A or infliximab may be used in combination with azathioprine and corticosteroids. Interferon-alfa, alone or in combination with corticosteroids, appears to be a second choice in this eye disease.

Last updated on 05-01-20

Where To Start

JHU Vasculitis Ctr - Behcet's disease

The Johns Hopkins Vasculitis Center website provides information about different types of vasculitis. Click on the link to access information about Behcet's disease.

Last updated on 04-27-20

Name: Vasculitis Foundation P.O. Box 28660
Kansas City, MO, 64188, United States
Phone: +1-816-436-8211 Toll Free: 1-800-277-9474 Fax : +1-816-656-3838 Email: Url:
Name: American Behcet's Disease Association ABDA PO Box 80576
Rochester, MN, 48308, United States
Phone: +1-631-656-0537 Toll Free: 1-800-723-4238 or 1-800-7-BEHCET (Hotline) Email: Url:
Name: Behcet's Organisation Worldwide Head Office PO Box 27
Watchet Somerset TA23 0YJ
United Kingdom
Email: Url:
Name: American Autoimmune Related Diseases Association (AARDA) 22100 Gratiot Avenue
Eastpointe, MI, 48021, United States
Phone: 586-776-3900 Toll Free: 800-598-4668 Fax : 586-776-3903 Email: Url:
Name: FMF & AID Global Association Schüracherstrasse 25a 8306 Brüttisellen
Phone: +41 76 415 4010 Email: Url:

Note, these links are external searches against the National Laboratory of Medicine's drug database. You may need to adjust the search if there are no results found.

Drug Name Generic Name
Otezla apremilast

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