What is Behcet’s Disease (Adamantiades-Behcet’s Disease; Silk Road Disease)

Behçet’s disease, also known as Adamantiades–Behçet’s disease or “silk road disease“, is a type of vasculitis. It is a rare, chronic, lifelong disorder that involves inflammation of blood vessels throughout the body. Behçet’s disease is a multi-system disease; it may involve any organ in the body including the central nervous system, resulting in memory loss and impaired speech, balance, and movement.

Statistics on Behcet’s Disease (Adamantiades-Behcet’s Disease; Silk Road Disease)

Behçet’s disease is common in the Middle East, Asia, and Japan, but rare in the United States. Behçet’s disease is most common along the “Old Silk Route”, which spans the region from Japan and China in the Far East to the Mediterranean Sea, including countries such as Turkey and Iran. In Japan, the current prevalence is 10-15 per 100,000; in Turkey, it is 80-300 per 100,000.

Behçet’s disease is most common among patients aged 15-45 years.

Risk Factors for Behcet’s Disease (Adamantiades-Behcet’s Disease; Silk Road Disease)

Behçet’s disease is one of the few forms of vasculitis (inflammation of blood vessels) in which there is a known genetic predisposition. The presence of a specific gene is a risk factor for this disease. However, it must be emphasised that presence of the gene in itself is not enough to cause Behçet’s disease; many people possess the gene, but relatively few develop Behçet’s disease. Despite the predisposition to Behçet’s disease conferred by this particular gene, familial cases make up only 5% of cases. It is believed that other factors (perhaps more than one) play a role. Possibilities include infections and other environmental exposures.

Progression of Behcet’s Disease (Adamantiades-Behcet’s Disease; Silk Road Disease)

Behçet’s disease initially is an inflammatory disease, but in severe cases, or if left untreated, the effects of the disease may include blindness, stroke, swelling of the spinal cord, and intestinal complications.

Symptoms of Behcet’s Disease (Adamantiades-Behcet’s Disease; Silk Road Disease)

Possible symptoms of Behçet’s disease include:

  • Painful oral lesions;
  • Memory loss, personality changes and speech impairment;
  • Instability;
  • Ocular symptoms such as blurred vision, periorbital pain, photophobia, eye redness and excessive tear production;
  • Headache;
  • Incontinence;
  • Inflammatory arthritis with warmth, redness, and swelling around the joint;
  • Back pain;
  • Diarrhoea, flatulence and vomiting.

How is Behcet’s Disease (Adamantiades-Behcet’s Disease; Silk Road Disease) Diagnosed?

There is no specific test for Behçet’s disease. Consequently, the diagnosis is based on the clinical signs and symptoms that are compatible with the disease – such as the presence of oral/genital ulcers, uveitis and skin lesions, etc.

An international group of physicians has established a set of guidelines to aid in the classification of Behçet’s patients for the purpose of conducting research in the disease. The criteria put forth by the group include recurrent oral ulceration (at least three occasions in a year). In addition, a patient must also meet two of the following four criteria for Behçet’s disease: recurrent genital ulcerations, eye lesions (uveitis or retinal vasculitis), skin lesions (erythema nodosum, lesions, acne), and or positive “pathergy test”.

The pathergy test is a simple test in which the forearm is pricked with a small, sterile needle. Occurrence of a small red bump or pustule at the site of needle insertion constitutes a positive test. Although a positive pathergy test is helpful in the diagnosis of Behcet’s, only a minority of Behçet’s patients demonstrates the pathergy phenomenon (i.e., have positive tests). Patients from the Mediterranean region are more likely to demonstrate pathergy. In addition, other conditions can occasionally result in positive pathergy tests, so the test is not 100% specific.

Prognosis of Behcet’s Disease (Adamantiades-Behcet’s Disease; Silk Road Disease)

Behçet’s disease is a lifelong disorder that comes and goes. Permanent remission of symptoms has not been reported. Death from Behçet’s disease is rare.

How is Behcet’s Disease (Adamantiades-Behcet’s Disease; Silk Road Disease) Treated?

Treatment for Behçet’s disease is symptomatic (according to symptoms) and supportive. Medication may be prescribed to reduce inflammation and/or regulate the immune system. Immunosuppressive therapy may be considered.

Behcet’s Disease (Adamantiades-Behcet’s Disease; Silk Road Disease) References

  1. Vasculitis [online]. Baltimore, MD: Johns Hopkins University; 2003 [cited 1 December 2003]. Available from: [URL link]
  2. Behcet’s syndrome [online]. Bethesda, MD: MedlinePlus; 2003 [cited 1 December 2003]. Available from: [URL link]
  3. Behcet’s disease [online]. Bethesda, MD: National Institute of Neurological Disorders and Stroke; 2003 [cited 1 December 2003]. Available from: [URL link]