|
|
Behçet's disease (BD, or Behçet's syndrome) is a rare, multisystem disease with chronic inflammation of blood vessels (i.e. vasculitis). It is also known as Silk Road Disease.
| Contents |
History
Behçet's disease is named after Hulusi Behçet (1889-1948), the Turkish dermatologist and scientist who first recognized the syndrome in one of his patients in 1924 and reported his research on the disease in Journal of Skin and Veneral Diseases in 1936. The name (Morbus Behçet) was formally adopted at the International Congress of Dermatology in Geneva in September 1947.
Benedikt Adamandiades, a Greek ophthalmologist, reportedly described the disease six years before Behçet did. Nevertheless, only Greek scientists refer to the disease Adamantiades-Behçet's or even Adamantiades' disease.
Pathology
Behçet's disease is believed to be caused by an over-active immune system which, without any apparent infections, produces recurrent outbreaks of inflammation in small blood vessels. As a result, symptoms can occur anywhere in the body with a blood supply. Common symptoms include mouth ulcers, sore genitals and eye inflammation, and arthritis in older patients, mostly painful but not life-threatening conditions. However, some patients may be unable to work because of the pain and the impaired vision and mobility. In some severe cases, uncontrolled inflammation may lead to blindness, intestinal complications, stroke, and even meningitis, which can become fatal.
This disease usually first strikes patients in their 20s and 30s. Then, it becomes a lifelong disorder that proceeds in a series of remissions (periods of apparent good health, usually a few months) and exacerbations (periods of active disease and progression, can be a week to a month). Complete remission is rare.
Treatment
There is no cure yet. Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system.
Epidemiology
Behçet's disease is considered more prevalent in the areas surrounding the old silk trading routes in the Middle East and in Central Asia. Thus, it is sometimes known as Silk Road Disease. However, this disease is not restricted to people from these regions.
An estimated 15,000 to 20,000 Americans have been diagnosed with this disease. In the UK, it is estimated to have about 2 cases for every 100,000 people.
Males are affected more frequently than females.
External links
- Signs & Symptoms of Behçet's disease (http://www.hulusibehcet.net/manifest.htm) (with pictures)
- American Behçet's Disease Association (http://www.behcets.com/home.ivnu)
- Behçet's Syndrome Society (http://www.behcets-society.fsnet.co.uk/index.html) (UK)
- International Society for Behçet's Disease (http://www.behcet.ws/)
- Behçet's syndrome (http://www.whonamedit.com/synd.cfm/1863.html) (Whonamedit.com )fr:Maladie de Behçet