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Genetic and Rare Diseases Information Center (GARD)

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Behcet's disease


Other Names for this Disease

  • BD
  • Behcet disease
  • Behcet syndrome
  • Behcet's syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Your Question

I have been diagnosed with Behcet's disease. Although I have other symptoms, my eyes have been causing me the most trouble. Please tell me more about the ocular issues with Behcet's and how they might be treated.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is Behcet's disease?

Behcet's disease is a chronic multisystem inflammatory disorder characterized by ulcers affecting the mouth and genitals, various skin lesions, and abnormalities affecting the eyes.[1][2] 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.[2] The exact cause of Behcet's disease is unknown.[1][2][3]  Treatment is symptomatic and supportive.[2]
Last updated: 12/4/2009

What are the signs and symptoms of Behcet's disease? 

Symptoms of Behcet's disease include recurrent ulcers in the mouth (resembling canker sores) and on the genitals, and eye inflammation (uveitis). The disorder may also cause various types of skin lesions, arthritis, bowel inflammation, meningitis (inflammation of the membranes of the brain and spinal cord), and cranial nerve palsies. Behcet's is a multi-system disease; it may involve all organs and affect the central nervous system, causing memory loss and impaired speech, balance, and movement.[1][2] The effects of the disease may include blindness, stroke, swelling of the spinal cord, and intestinal complications.[2]
Last updated: 8/31/2011

My eyes are giving me a great deal of trouble. What else can you tell me about Behcet's 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's disease. This symptom is more common among men than women and typically begins within 2 years of the first symptoms.[3] 

Behcet’s 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.[4]   

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.[3]   

More detailed information about the ocular findings in Behcet's disease can be found by clicking here.

Last updated: 8/26/2009

What causes Behcet's disease?

The exact cause of Behçet's 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.[3]

Behçet's disease is not contagious; it is not spread from one person to another. Researchers think that two factors are important for a person to get Behçet's 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.[3]

Last updated: 8/31/2011

How might Behcet's disease be treated?

Although there is no cure for Behçet's 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.[3]

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.[3]

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's disease.[3]

Last updated: 8/31/2011

How are the eye symptoms in Behcet's disease managed?

In terms of the ocular (eye) symptoms in Behcet's disease, the goals of therapy are to suppress inflammation, to reduce the frequency and severity of recurrences, and to minimize involvement of the retina. To be effective, treatment must be started early. Extent of involvement and severity of disease determine the choice of medication. Treatment options include:[5]

  • Systemic corticosteroids which effectively suppress all phases of ocular involvement in Behçet's disease. Although these drugs do not prevent visual deterioration, systemic corticosteroid therapy may still be helpful, especially when used in concurrence with other immunosuppressive agents.
  • Cytotoxic agents such as Chlorambucil 
  • Cyclosporine which, at a dose of 5 mg/kg per day, may effectively limit the frequency of ocular inflammatory attacks in up to 70% of patients with Behçet's disease, even in those whose symptoms were previously refractory to treatment.
  • Colchicine

More detailed information about the management of the ocular symptoms of Behcet's disease can be accessed by clicking here.

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.
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed

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: 8/26/2009

What is the prognosis for individuals with Behcet's disease?

Most people with Behçet's 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's disease.[3]
Last updated: 12/4/2009

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)
Information Office
31 Center Drive MSC 2510
Bethesda, MD 20892-2510
Telephone:  301-496-5248
E-mail: 2020@nei.nih.gov
Web site:  http://www.nei.nih.gov/
Web page on Behcet's disease: http://www.nei.nih.gov/health/behcets/

Last updated: 8/26/2009

References
Other Names for this Disease
  • BD
  • Behcet disease
  • Behcet syndrome
  • Behcet's syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.