The following information may help to address your question:
What is 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.
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.
Last updated: 11/2/2016
What are the signs and symptoms of Behçet disease?
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:
- Sores inside the mouth or genitals: The earliest symptom of Behçet disease is usually painful canker round or oval sores with reddish borders on the mucous membranes that line the mouth (aphthous stomatitis) or in the skin of the genitalia. They may be shallow or deep and may be single or multiple lesions that typically heal within a few days, up to a week or more, without scarring, but frequently recur.
- Eye problems: Symptoms may include inflammation of the back of the eye (posterior uveitis); inflammation of the anterior chamber (anterior uveitis or iridocyclitis); inflammation of the iris accompanied by pain, tearing (lacrimation); and accumulation of pus (hypopyon iritis). The retina may become inflamed resulting in blurred vision, abnormal sensitivity to light (photophobia), and/or inflammation of the thin membranous layer of blood vessels behind the retina (chorioretinitis). Repeated recurrences may result in the partial loss of vision (decreased visual acuity) or complete blindness if the disease is uncontrolled. In some cases, eye abnormalities may be the first symptom of Behçet disease or they may not develop until several years after the sores of the mouth.
- Pus-filled lesions and other problems on the skin: Some affected individuals, especially females, may develop lesions that resemble those of erythema nodosum, a skin disorder characterized by the formation of tender, reddish, inflammatory nodules on the front of the legs. These nodules disappear on their own (spontaneously) sometimes leaving faint scars or discoloration (pigmentation). Some people with Behçet disease may develop small eruptions that resemble acne (acneiform eruptions) and/or inflammation that mistakenly appear to affect the hair follicles on the skin (pseudofolliculitis).
- Pain in the joints (50% of cases): Affected individuals have pain (arthralgia) and swelling in various joints (knees, wrists, elbows and ankles), before, during, or after the onset of the other symptoms.
- Recurring ulcers in the digestive tract: Symptoms vary from mild abdominal discomfort to severe inflammation of the large intestine and rectum accompanied by diarrhea or bleeding.
- Problems of the central nervous system (10%-20% of the cases): These symptoms usually appear months or years after the initial symptoms of Behçet disease. Recurring attacks of inflammation involving the brain (parenchymal Neuro-Behçet) or the membranes that surround the brain or spinal cord (meningitis) can result in neurological damage. Symptoms may include headache, cranial nerve palsies, the inability to coordinate voluntary movement (cerebellar ataxia), impaired muscle movements of the face and throat, stroke, memory loss and/or, rarely, seizures.
- Inflammation of the blood vessels (vasculitis): Involvement of small vessels is thought to drive many of the problems that the disorder causes. In some instances inflammation of the large veins, particularly those in the legs may occur along with the formation of blood clots (thrombophlebitis). The walls of an involved artery may bulge forming a sac (aneurysm). In very rare cases, blood clots from the veins travel to the lungs (pulmonary emboli) resulting in episodes of chest pain, coughing, difficult or labored breathing (dyspnea), and coughing up blood (hemoptysis).
It is especially important to identify Behçet disease when there is ocular, central nervous system or large blood vessel involvement as manifestations are usually the most serious.
Last updated: 11/2/2016
My eyes are giving me a great deal of trouble. What else can you tell me about Behçet disease and the eyes?
Uveitis (yoo-vee-EYE-tis) involves inflammation of the middle or back part of the eye (the uvea), including the iris
, and occurs in more than half of all people with Behçet disease. This symptom is more common among men than women and typically begins within two years of the first symptoms.
Behçet disease may cause either anterior uveitis (inflammation in the front of the eye) or posterior uveitis (inflammation in the back of the eye), and sometimes causes both at the same time (panuveitis). Anterior uveitis results in pain, blurry vision, light sensitivity, tearing, or redness of the eye. Posterior uveitis may be more dangerous and vision–threatening because it often causes fewer symptoms while damaging a crucial part of the eye — the retina.
Because partial loss of vision or blindness can result if the eye frequently becomes inflamed, patients should report these symptoms to their doctor immediately. An ophthalmologist should be involved in treatment of the eye inflammation.
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 eye disease.
More detailed information about the ocular findings in Behçet disease is available.
Last updated: 11/2/2016
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. 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. Second, something in the environment, possibly a bacterium or virus, might trigger or activate the disease in susceptible people.
Last updated: 11/2/2016
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.
, 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: 11/2/2016
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: 11/2/2016
Who can I contact for more information about the ocular aspects of Behcet's disease?
For more information on the ocular aspects of Behcet's disease, contact The National Eye Institute (NEI), part of the National Institutes of Health (NIH).
National Eye Institute (NEI)
31 Center Drive MSC 2510
Bethesda, MD 20892-2510
Webpage on Behcet's disease: http://www.nei.nih.gov/health/behcets/
You can also find relevant journal articles on the treatment of Behcet uveitis through a service called PubMed, a searchable database of medical literature. Information on finding an article and its title, authors, and publishing details is listed here. Some articles are available as a complete document, while information on other studies is available as a summary abstract. To obtain the full article, contact a medical/university library (or your local library for interlibrary loan), or order it online using the following link. Using "Behcet uveitis AND treatment" as your search term should locate articles. To narrow your search, click on the "Limits" tab under the search box and specify your criteria for locating more relevant articles. Click here to view a search.
The National Library of Medicine (NLM) Web site has a page for locating libraries in your area that can provide direct access to these journals (print or online). The Web page also describes how you can get these articles through interlibrary loan and Loansome Doc (an NLM document-ordering service). You can access this page at the following link http://nnlm.gov/members/. You can also contact the NLM toll-free at 888-346-3656 to locate libraries in your area.
Last updated: 11/29/2016
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