Behcet's Disease Support Group

Behçet's disease (also known as Adamantiades-Behçet's disease), is a chronic condition due to disturbances in the body’s immune system. This system, which normally protects the body against infections by producing controlled inflammation, becomes overactive and produces unpredictable outbreaks of exaggerated inflammation. This extra inflammation affects blood vessels, usually the small ones. As a result, symptoms occur wherever there is a patch of inflammation, and can be anywhere where there is a blood supply.

The symptoms of Behçet's disease are believed to be caused by an over-active immune system which, without any apparent infections, produces recurrent outbreaks of inflammation in small blood vessels. 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 be fatal.

This disease usually first strikes patients in their 20s and 30s. It then becomes a fluctuating lifelong disorder with a series of remissions and exacerbations which can be from days to months. Complete remission is rare.

Current treatment is aimed at easing the symptoms, reducing inflammation, and controlling the immune system. Anti-TNF therapy such as infliximab has shown promise in treating the uveitis associated with the disease (Sfikakis et al, 2001; Sfikakis, 2002). Another Anti-TNF agent, Etanercept, may be useful in patients with mainly skin and mucosal symptoms (Melikoglu, 2005).

Interferon alfa-2a may also be an effective alternative treatment, particularly for the genital and oral ulcers (Alpsoy, 2002) as well as ocular lesions (Kotter, 2003). Azathioprine, when used in combination with interferon alfa-2b also shows promise (Hamuryudan, 2002), and Colchicine can be useful for treating some genital ulcers, erythema nodosum, and arthritis in women, and arthritis in men (Yurdakul et al, 2001).

Thalidomide has also been used due to its immune-modifying effect (Hamuryudan et al, 1998). Dapsone and rebamipide have been shown, in small studies, to have beneficial results for mucocutaneous lesions (Matsuda et al, 2003;Sharquie et al, 2002).