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Don’t fight Behçet disease alone.
Find your community on the free RareGuru App.Behçet disease is a chronic multisystem inflammatory disorder characterized by ulcers affecting the mouth and genitals, various skin lesions, and abnormalities affecting the eyes. In some people, the disease also results in arthritis (swollen, painful, stiff joints), skin problems, and inflammation of the digestive tract, brain, and spinal cord. Although it can happen at any age, symptoms generally begin when individuals are in their 20s or 30s. The disease is common in Japan, Turkey and Israel, and less common in the United States. The exact cause of Behçet disease is still unknown, but it is thought that it is an autoimmune disease, where the abnormal immune activity is triggered by exposure to an environmental agent (such as an infection) in people with a genetic predisposition to develop the disease. Research show that people with Behçet’s disease, especially those of Middle Eastern and Asian descent, have an increased frequency of certain “human leukocyte antigens” (HLAs), specifically HLA-B51, than the general population, which may increase the risk (predispose) to have the disease.
Treatment is symptomatic and supportive. Research is being conducted on the use of interferon-alpha and with agents which inhibit tumor necrosis factor (TNF) for the treatment of Behçet disease. Behçet disease is a lifelong disorder that comes and goes. Spontaneous remission over time is common for individuals with Behçet disease but permanent remission of symptoms has not been reported.
Source: GARD Last updated on 05-01-20
The signs and symptoms of Behçet disease include recurrent ulcers in the mouth and on the genitals, and eye inflammation (uveitis). It usually begins when people are in their 20s or 30s, although it can happen at any age. It tends to occur more often in men than in women. Behçet disease is a multi-system disease and it may involve all organs of the body. Signs and symptoms may include:
It is especially important to identify Behçet disease when there is ocular, central nervous system or large blood vessel involvement because these as manifestations are usually the most serious.
Last updated on 05-01-20
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:
Last updated on 05-01-20
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
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
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
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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