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Disease Mechanisms

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Book cover Behçet Syndrome

Abstract

An infectious agent is possibly required to trigger the inflammation in Behçet syndrome (BS). 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 T or B cells with mainly a pro-inflammatory and Th1/Th17 type of cytokine profile. Adaptive immune responses against various autoantigens such as heat shock proteins are shown to be expressed, especially by the nature of the peripheral blood cells of BS patients; however, their pathogenic role is still not clear. Among genetic associations, HLA-B∗51 is the most clearly defined, and “MHC-I-opathies” are suggested as a unified concept of spondyloarthropathies and BS. Organ-specific pathogenic factors such as venous thrombotic tendency or the role of male gender in disease severity are also not clarified yet.

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Authors and Affiliations

  1. Department of Internal Medicine, Division of Rheumatology, Marmara University, School of Medicine, Istanbul, Turkey

    Haner Direskeneli

  2. Department of Physiology, Istanbul University, Istanbul Medical Faculty, Istanbul, Turkey

    Güher Saruhan-Direskeneli

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  1. Haner Direskeneli
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Editors and Affiliations

  1. School of Medicine, New York University, New York, NY, USA

    Yusuf Yazici

  2. Department of Internal Medicine, Istanbul University, Istanbul, Turkey

    Gulen Hatemi

  3. Department of Rheumatology, Istanbul University, Istanbul, Turkey

    Emire Seyahi

  4. Department of Medicine, Academic Hospital, Istanbul, Turkey

    Hasan Yazici

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Direskeneli, H., Saruhan-Direskeneli, G. (2020). Disease Mechanisms. In: Yazici, Y., Hatemi, G., Seyahi, E., Yazici, H. (eds) Behçet Syndrome. Springer, Cham. https://doi.org/10.1007/978-3-030-24131-5_15

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