What causes Behçet disease?

Updated: Dec 21, 2020
  • Author: Nicole Davey-Ranasinghe, MD; Chief Editor: Herbert S Diamond, MD  more...
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Answer

The specific etiology of Behçet disease remains elusive but, as described in Pathophysiology, the interplay between infectious-agent exposure and genetic factors may have a role. An environmentally triggered hyperactive primed state of autoimmunity ensues, resulting in two types of vascular damage. The first is vasculitic lesions that may be widespread. Sequelae depend on the various organ systems affected.

Some of the pathologic changes are not vasculitis but due to thrombosis and/or clot formation caused by the development of a hypercoagulable state. The mechanism is still undetermined; however, studies have demonstrated excessive thrombin formation and the potential role of impaired fibrinolytic kinetics in the generation of the hypercoagulable/prothrombotic state. Pathologic activation of the procoagulant cascade via endothelial injury has also been demonstrated in patients with Behçet disease. [26, 27]


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