Behçet Syndrome
Behçet Syndrome
Purpose of Review: Interest in Behçet syndrome (BS), although the condition is rare in many parts of the world, is increasing as judged by the growing number of related publications and scientific meetings.
Recent Findings: Recent work reconfirmed the following without further insight into their meaning in pathogenesis: males have a more severe course; one third of the patients have a thrombophilia; papulopustular lesions and arthritis go together; γδ T are increased; and HLA B51 is still the main genetic association. Presence of increased levels of antibodies to Saccharomyces cerevisiae and the proposal of α-enolase as the target antigen of antiendothelial cell antibodies, known to be present also in BS, are observations that might help in delineating the unknown pathogenesis. For management, preliminary yet promising experience with the use of anti-tumor necrosis factor agents is being reported.
Summary: When compared with 2 decades ago, we know considerably more and can do more about BS.
Behçet syndrome (BS) is a systemic vasculitis of small and large vessels of unknown origin. With oral ulceration as its hallmark and sight-threatening uveitis as its main morbidity, it has, again for unknown reasons, a unique geographic distribution, attributed by some to the old Silk Road. There is an ever-increasing interest in BS, not only because of its being an important cause of morbidity and mortality in areas where it is prevalent, but also generally as a unique model of vasculitis. We review the highlights of the most recent work on BS.
Purpose of Review: Interest in Behçet syndrome (BS), although the condition is rare in many parts of the world, is increasing as judged by the growing number of related publications and scientific meetings.
Recent Findings: Recent work reconfirmed the following without further insight into their meaning in pathogenesis: males have a more severe course; one third of the patients have a thrombophilia; papulopustular lesions and arthritis go together; γδ T are increased; and HLA B51 is still the main genetic association. Presence of increased levels of antibodies to Saccharomyces cerevisiae and the proposal of α-enolase as the target antigen of antiendothelial cell antibodies, known to be present also in BS, are observations that might help in delineating the unknown pathogenesis. For management, preliminary yet promising experience with the use of anti-tumor necrosis factor agents is being reported.
Summary: When compared with 2 decades ago, we know considerably more and can do more about BS.
Behçet syndrome (BS) is a systemic vasculitis of small and large vessels of unknown origin. With oral ulceration as its hallmark and sight-threatening uveitis as its main morbidity, it has, again for unknown reasons, a unique geographic distribution, attributed by some to the old Silk Road. There is an ever-increasing interest in BS, not only because of its being an important cause of morbidity and mortality in areas where it is prevalent, but also generally as a unique model of vasculitis. We review the highlights of the most recent work on BS.