Behcet’s is a form of vasculitis.
It commonly involves small to medium sized blood vessels. It is a condition more common around the Mediterranean, Middle East and Far East. Males and females are affected equally but males tend to have more severe disease.
How common is Behcet’s Disease?
Behcet’s is rare in the United States. In the US, it is estimated that around 3-5/100,000 people have Behcet’s. In Turkey, where it is very common, as many as 1 in 250 people have it. It is a leading cause of blindness in Israel and Japan.
Who gets Behcet’s Disease?
Males and females are equally affected. Behcet’s is more common among people who live around the Mediterranean, Middle East and Far East, and to a lesser extent, among the descendants of people from these areas living in other parts of the world. Patients are usually 20 to 30 years of age when they develop it, and the condition is rare in children and the elderly.
The most common symptoms are oral and genital ulcers. Other skin lesions range from acne to erythema nodosum type nodular lesions. Half of the patients tend to develop inflammation in the eye which can lead to blindness if not treated. Less common is involvement of blood vessels leading to blood clots, aneurysms and the central nervous system. Gastrointestinal involvement is more common in the United States and the Far East than around the Mediterranean. Arthritis with joint swelling can occur and usually involves larger joints.
Behcet’s is diagnosed clinically. There are no laboratory or imagining tests that are specific for the diagnosis of this disease. The work up with laboratory tests and imaging is done to rule out other conditions that may mimic the symptoms of Behcet’s. HLA B51 is a genetic marker that is seen more commonly in patients with Behcet’s, however it is also seen in up to 20% of the general population, so by itself, presence of this gene cannot help to establish the diagnoses. The diagnosis depends on a good physical examination, a detailed history and presence of the typical symptoms and signs of the disease, keeping in mind that there are a number of other diseases which can mimic Behcet’s.
Behcet’s is a treatable condition. The treatment depends on the extent of symptoms. Eye involvement almost always requires the use of immunosuppressant medications and corticosteroids to prevent vision loss. Mucocutaneous involvement including mouth sores can often be treated topically or with milder medications by mouth such as colchicine. For vascular and gastrointestinal involvement immunosuppressants are used in addition to corticosteroid therapy. Most patients need 2-5 years of treatment, and most patients have lessening of the severity of their symptoms and may not need any medications and/or have no or minimal symptoms with the passage of time.
What are the complications of Behcet’s Disease?
The most common serious complication is vision loss, especially if the eye involvement is not treated in a timely manner. Even with treatment, some patients may lose vision. Mucocutaneous involvement usually resolves with no after effects. The arthritis of Behcet’s is rarely deforming.
What is the prognosis?
The natural history of Behcet’s is different from most rheumatologic conditions in that most patients have lessening of the severity of their symptoms and up to 60-70% may not need any medications and/or have no or minimal symptoms with time. Females tend to have milder disease than males.
What is new in Behcet’s Disease?
New biologic agents developed for rheumatoid arthritis and similar conditions are being tested in Behcet’s. In addition, research into understanding the interaction between genetic risk factors and environment is ongoing and may shed light on the pathogenesis of this condition.
Revision: September 2012
The Vasculitis Foundation gratefully acknowledges Dr. Yusef Yazici, Hospital for Joint Disease and Medicine, New York, N.Y., for his expertise and contribution in compiling this information.
Click here to download the Behcet’s Disease Brochure in PDF format
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