Behçet's Disease of the Eye

The information provided in this Resource Guide was developed by the National Eye Institute to help patients and their families in searching for general information about Behçet's disease. An eye care professional who has examined the patient's eyes and is familiar with his or her medical history is the best person to answer specific questions.

Other Names

Adamantiades

What is Behçet's disease?

Behçet's disease is an autoimmune disease that results from damage to blood vessels throughout the body, particularly veins. In an autoimmune disease, the immune system attacks and harms the body's own tissues.

What causes Behçet's disease?

The exact cause is unknown. It is believed that an autoimmune reaction may cause blood vessels to become inflamed, but it is not clear what triggers this reaction.

What are the symptoms of Behçet's disease?

Behçet's disease affects each person differently. The four most common symptoms are mouth sores, genital sores, inflammation inside of the eye, and skin problems. Inflammation inside of the eye (uveitis, retinitis, and iritis) occurs in more that half of those with Behçet's disease and can cause blurred vision, pain, and redness.

Other symptoms may include arthritis, blood clots, and inflammation in the central nervous system and digestive organs.

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How is Behçet's disease treated?

There is no cure for Behçet's disease. Treatment typically focuses on reducing discomfort and preventing serious complications. Corticosteroids and other medications that suppress the immune system may be prescribed to treat inflammation.

What is the prognosis for someone with Behçet's disease?

Behçet's is a chronic disease that recurs. However, patients may have periods of time when symptoms go away temporarily (remission). How severe the disease is varies from patient to patient. Some patients may live normal lives, but others may become blind or severely disabled.

NEI-Supported Research

A Study to Investigate the Safety and Efficacy of HAT to Treat the Ocular Complications Related to Behçet's Disease

The National Eye Institute is evaluating the safety and effectiveness of Zenapax in controlling recurrent eye inflammations associated with Behçet's disease. Zenapax was previously studied in 10 patients with uveitis with positive results. The patients were able to reduce the other medicines they were taking with minimal side effects. This study is currently recruiting patients. For additional information, please contact NIH Patient Recruitment and Public Liaison Office at 1-800-411-1222.

Other Resources

The following organizations may be able to provide additional information on Behçet's disease:

National Institute of Arthritis and Musculoskeletal and Skin Diseases (NIAMS)
1 AMS Circle
Bethesda, MD 20892-3675
(301) 496-8188
http://www.nih.gov/niams
Conducts and supports research on the many forms of arthritis and diseases of the musculoskeletal system (bones) and the skin. Provides information on Behçet's disease.

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In addition to our free knowledge base, BiotechWatch provides the following additional services: Premium Database, for in-depth coverage of biotechnology companies in the sector, giving the user the ability to search and sort based on more than twenty parameters. TrialView Database, which offers disease-specific and biotechnology sector-wide clinical trial information. Make your View Count - at BiotechWatch's You Too Can Be An Analyst. Vote on the success of upcoming biotech events. Visit the Biotech Forum, where you can exchange ideas and generate new biotech investments.

American Behçet's Disease Association
P.O. Box 15247
Chattanooga, TN 37415-0240
1-800-723-4238
http://www.behcets.com
Provides support and information to people with Behçet's disease, publishes a quarterly newsletter, distributes patient pamphlets, and coordinates a pen pal/phone pal network. Provides physician referrals, coordinates a network of local support groups, and holds an annual international conference.

National Organization for Rare Disorders, Inc. (NORD)
P.O. Box 8923
New Fairfield, CT 06812-8923
(203) 746-6481
1-800-999-6673
http://www.rarediseases.org
Provides information about rare disorders and connects families with similar disorders with each other for mutual support. Serves people with orphan diseases who are not otherwise represented. Maintains information on Behçet's disease in the Rare Disease Database.

For additional information, you may also wish to contact a local library.

Medical Literature

Below is a sample of the citations available through MEDLINE/PubMed, a service of the National Library of Medicine. MEDLINE/PubMed provides access to over 11 million medical literature citations from 1966 to the present and includes links to many sites providing full text articles and other related resources. You can conduct your own free literature search by accessing MEDLINE through the Internet at http://medlineplus.nlm.nih.gov/hinfo.html. You can also get assistance with a literature search at a local library.

To obtain copies of any of the articles listed below, contact a local community, university, or medical library. If the library you visit does not have a copy of a desired article, you may usually obtain it through an inter-library loan.

Please keep in mind that articles in the medical literature are usually written in technical language. We encourage you to share any articles you order with a health care professional who can help you understand them.

Meador R, Ehrlich G, Von Feldt JM: Behçet's disease: immunopathologic and therapeutic aspects. Current Rheumatology Reports 4(1):47-54, February 2002.
Behçet's disease (BD) is a systemic inflammatory disease of unknown etiology. The disease is strongly associated with the human leukocyte antigen (HLA) B51. BD has a chronic course with periodic exacerbations and progressive deterioration. There are no specific diagnostic laboratory tests, although recurrent oral ulceration is an obligatory manifestation for diagnosis. The treatment, which includes local, systemic, or surgical therapies, is based on the severity of the illness; the most appropriate management requires a multidisciplinary approach. This paper summarizes all aspects of BD with particular emphasis on the latest immunologic and treatment aspects.

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In addition to our free knowledge base, BiotechWatch provides the following additional services: Premium Database, for in-depth coverage of biotechnology companies in the sector, giving the user the ability to search and sort based on more than twenty parameters. TrialView Database, which offers disease-specific and biotechnology sector-wide clinical trial information. Make your View Count - at BiotechWatch's You Too Can Be An Analyst. Vote on the success of upcoming biotech events. Visit the Biotech Forum, where you can exchange ideas and generate new biotech investments.

Ehrlich GE: Behçet's Disease: An Update. University of Pennsylvania School of Medicine. Comprehensive Therapy 25(4):216-20, April 1999.
This article reviews current therapies for Behçet's disease, although no diagnostic laboratory test or cure has been found. Genetic studies have identified those most at risk, and newer molecular biologic investigations further clarify its etiology and shed light on potential triggers.

George RK, Chan CC, Whitcup SM, Nussenblatt RB: Ocular Immunopathology of Behçet's Disease. Madigan Army Medical Center, Tacoma, Washington. Survey of Ophthalmology 42(2):157-62, 1997.
This article describes a patient who developed progressive, severe, recurrent iridocyclitis in both eyes, retinal vasculitis, and hemorrhagic infarction of the retina that led to blindness despite immunosuppressive therapy. Histopathology revealed marked nongranulomatous uveitis with a predominantly CD4+ T-lymphocytic infiltration, as well as B-cell and plasma cell aggregation. Extensive expression of adhesion molecules was found on vascular endothelial cells. This finding suggests that adhesion molecules play an important role in the vasculitic process that is the hallmark of Behçet's disease. The ocular pathology and the therapeutic approach to Behçet's disease are briefly reviewed as well.

The National Eye Institute, part of the National Institutes of Health, is the Federal government's principal agency for conducting and supporting vision research. Inclusion of an item in this Information Resource Guide does not imply the endorsement of the National Eye Institute or the National Institutes of Health.