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Disease Mechanisms

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Behçet’s Syndrome

Abstract

An infectious agent is possibly required to trigger the inflammation in Behçet’s disease (BD). Innate immune system through neutrophils is activated early and aggressively. However, unlike classical autoinflammatory disorders, an adaptive response is also possibly sustained through bacterial persistence or autoantigen-activated dendritic cells and T or B cells with mainly a pro-inflammatory and Th1 type of cytokine profile. Adaptive immune responses against various auto-antigens such as heat-shock proteins, retinal-S antigen, and alpha-tropomyosin are shown to be present especially in the peripheral blood cells of BD patients; however, their pathogenic roles are not yet clear. Among genetic associations with BD, HLA-B51 is the most clearly defined; however, except for “molecular mimicry” mechanisms leading to CD4+ T-cell responses to B51-associated peptides, other HLA-B27 association modeled pathogenetic pathways have not been explored in BD. Pathogenic mechanisms associated with disease severity such as male-gender and thrombosis, such as pro-thrombotic tendency, are also not clarified.

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  1. Department of Rheumatology, Marmara University Medical Faculty, Istanbul, Turkey

    Haner Direskeneli

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  1. Hospital for Joint Diseases, Behçet's Syndrome Center, NYU Medical Center, East 20th Street 246, New York, 10003, New York, USA

    Yusuf Yazıcı

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    Hasan Yazıcı

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Direskeneli, H., Saruhan-Direskeneli, G. (2010). Disease Mechanisms. In: Yazıcı, Y., Yazıcı, H. (eds) Behçet’s Syndrome. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-5641-5_14

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