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Behçet's disease

Krause, Ilana; Weinberger, Abrahamb

Current Opinion in Rheumatology: January 2008 - Volume 20 - Issue 1 - p 82–87
doi: 10.1097/BOR.0b013e3282f154d1
Systemic disorders with rheumatic manifestations: Edited by H. Ralph Schumacher, Frank Pessler and Lan X. Chen

Purpose of review To summarize recent scientific developments in the epidemiology, genetics, pathogenesis and treatment of Behçet's disease.

Recent findings Important genetic and immunologic studies were performed. Tumor necrosis factor-α-1031C allele was associated with disease susceptibility. Polymorphisms in interleukin-10, IL-8 and CD28 genes were also associated with Behçet's disease. Association with endothelial nitric oxide synthase gene polymorphism was confirmed but was ethnic related. Significant T helper type 1 immune reaction was reconfirmed in recent studies, especially during active phases, but T helper type 2 reaction may also play a role. Interleukin-12B heterozygosity is associated with Behçet's disease susceptibility and plays an important role in mediating T helper type 1 antistreptococcal immune response. Selenium binding protein may be a target antigen in Behçet's uveitis. Pathergy reaction is most frequently positive in the forearm; multiple needle pricks increase positive rate. Experience with anti-tumour necrosis factor therapy for various manifestations is increasing. Cyclosporin A treatment may be associated with new onset of neuro-Behçet. There is a high prevalence of headaches with moderate or severe disability. Cardiac manifestations include left ventricular dysfunction and coronary flow abnormalities. Anti-Saccharomyces cerevisiae antibodies may be especially common in intestinal Behçet's disease and are also increased in healthy relatives of patients.

Summary Considerable progress has been made, particularly in understanding the immunologic and genetic basis of the disease. The importance of novel serological markers and autoantigens merits further investigations.

aDepartment of Medicine E, Rabin Medical Center, Beilinson Hospital, Sackler Faculty of Medicine, Tel-Aviv University, Israel

bDepartment of Medicine B, Rabin Medical Center, Beilinson Hospital and Laboratory of Pathophysiology of the Joints, Felsenstein Medical Research Center, Sackler Faculty of Medicine, Tel-Aviv University, Israel

Correspondence to Ilan Krause, MD, Department of Medicine E, Rabin Medical Center, Beilinson Campus, Petah-Tiqva, 49100, Israel Fax: +972 3 9376512; e-mail:

© 2008 Lippincott Williams & Wilkins, Inc.