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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.
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.
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.
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.
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.
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.
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.
The following organizations may be able to provide additional information on Behçet's disease:
American Autoimmune Related Diseases Association, Inc. (AARDA)
22100 Gratiot Avenue
E. Detroit, MI 48021
1-800-598-4668 (Literature Requests)
Dedicated to bringing a national focus to autoimmune diseases, conditions caused by the body attacking itself. Provides information on eye-related diseases include Behçet's disease, Graves' disease, multiple sclerosis, myasthenia gravis, sarcoidosis, Sjögren's syndrome, temporal arteritis (giant cell arteritis), and uveitis.
American Behçet's Disease Association
P.O. Box 19952
Amarillo, TX 79114
Provides support and information to individuals with Behçet's disease, symptoms of which include ocular inflammation (uveitis). Publishes a quarterly newsletter and distributes patients pamphlets. Coordinates a pen pal/phone pal network. Provides physician referrals. Coordinates a network of local support groups. Holds an annual international conference.
National Eye Institute (NEI)
31 Center Drive MSC 2510
Bethesda, MD 20892-2510
Conducts and supports research on eye diseases and vision disorders. Offers free publications for the general public and patients.
National Institute of Arthritis, Musculoskeletal and Skin Diseases (NIAMS)
Building 31, Room 4C02
31 Center Drive MSC 2350
Bethesda, MD 20892-2350
Conducts and supports research on the many forms of arthritis and diseases of the musculoskeletal system (bones) and the skin. Distributes information on temporal arteritis (giant cell arteritis) and Behçet's disease. Also publishes Questions & Answers about Sjögren's Syndrome.
National Organization for Rare Disorders
55 Kenosia Avenue
P.O. Box 1968
Danbury, CT 06813-1968
(203) 797-9590 (TDD)
Acts as a clearinghouse for information about rare disorders. Fosters networks between families with similar disorders. Educates the general public and medical profession about the existence, diagnosis, and treatment of rare disorders.
Office of Rare Diseases, National Institutes of Health
6100 Executive Boulevard
Room 3B01, MSC 7518
Bethesda, MD 20892
Coordinates the Rare Disease Clinical Research Database (RDCRD). Offers information on more than 6,000 rare diseases, current research and clinical trials, and support groups.
For additional information, you may also wish to contact a local library.
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.
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.
This page was last modified in April 2005
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