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Pathogenesis of Behçet’s syndrome

Ellison L Smith, MD
Yusuf Yazici, MD
Section Editor
Peter A Merkel, MD, MPH
Deputy Editor
Monica Ramirez Curtis, MD, MPH


Behçet’s syndrome is an inflammatory disease characterized by recurrent oral aphthous ulcers and numerous potential systemic manifestations. These include genital ulcers; skin lesions; and ocular, neurologic, vascular, articular, and gastrointestinal disease.

Many, but not all, clinical manifestations of Behçet’s syndrome are believed to be due to vasculitis. Among the systemic vasculitides, Behçet’s syndrome is remarkable for its ability to involve blood vessels of all sizes (small, medium, and large) on both the arterial and venous sides of the circulation.

The etiology and pathogenesis of Behçet’s syndrome are discussed in this review. The clinical manifestations, diagnosis, and treatment of this disorder are presented separately. (See "Clinical manifestations and diagnosis of Behçet’s syndrome" and "Treatment of Behçet’s syndrome".)


Behçet’s syndrome is more common (and often more severe) along the ancient silk road, which extends from eastern Asia to the Mediterranean [1]. It is most common in Turkey (80 to 370 cases per 100,000), while the prevalence ranges from 13.5 to 20 per 100,000 in Japan, Korea, China, Iran, and Saudi Arabia [1]. Prevalence in Paris, France in 2003 was 7.1 per 100,000, with rates of 2.4 per 100,000 in those of European ancestry, of 34.6 per 100,000 in those of North African ancestry, and of 17.5 per 100,000 in those of Asian ancestry [2,3]. Estimates of prevalence in the United States and Europe have ranged from 0.12 to 7.5 per 100,000. Analysis of residents of Olmsted County, Minnesota over a 45-year period identified a prevalence of 5.2 per 100,000 [4]. It is somewhat more common in men in the eastern Mediterranean area and in women in north European countries, and it typically affects young adults 20 to 40 years of age [1]. Immigrant and refugee populations from areas of high Behçet's prevalence demonstrate increased risk of disease development [5].


The underlying cause of Behçet’s syndrome is unknown. As with other autoimmune diseases, the disorder may represent aberrant immune activity triggered by exposure to an agent, perhaps infectious, in patients with a genetic predisposition to develop the disease. Major disease mechanisms in Behçet’s syndrome include the following [6]:


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Literature review current through: Sep 2016. | This topic last updated: Nov 13, 2015.
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