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Behçet’s Syndrome pp 35–52Cite as

Epidemiology of Behçet’s Syndrome and Regional Differences in Disease Expression

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Abstract

Behcet’s syndrome (BS) is more prevalent along the ancient trading route known as the “Silk Road,” extending from the Mediterranean to the countries in the Far East, where the prevalence of HLA-B5(51) is more frequent in the healthy population. This suggests that the possible causative agent(s), including genetic factors such as HLA-B51, might have had spread along this route. Five cross-sectional and population-based field studies showed that the prevalence of BS was between 20 and 421 among 100,000 adolescent/adult population in Turkey. The lower two frequencies (20/105 and 80/105) come from Thrace, the European part. The disease is less common in Western Europe, USA, and in the rest of the world but perhaps not as rare as it had previously been thought. A recent study showed that BS was as frequent (7.1/105) as other vasculitic diseases – 9/105 for the combination of polyarteritis nodosa, microscopic polyangiitis, Wegener’s granulomatosis, and Churg–Strauss syndrome – in a suburb of Paris, France. We will review the epidemiological features of the field surveys of BS in Turkey and the rest of the globe. We also look at the regional differences of some of the clinical and laboratory features. Gastrointestinal disease is frequent in the patients from the Far East (especially Japan) while it is rather infrequent among those from Turkey. Moreover, a high frequency of both pathergy test positivity and being a carrier of HLA-B5(51) are observed in patients from Japan and the Mediterranean countries, in contrast to the patients from the United Kingdom and the USA.

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  1. Department of Medicine, Division of Rheumatology, Cerrahpasa Medical Faculty, University of Istanbul, Istanbul, Turkey

    Sebahattin Yurdakul

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  1. Sebahattin Yurdakul
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  2. Yusuf Yazıcı
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Correspondence to Sebahattin Yurdakul .

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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

  2. , Department of Medicine, University of Istanbul, Istanbul, 34452, Turkey

    Hasan Yazıcı

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Yurdakul, S., Yazıcı, Y. (2010). Epidemiology of Behçet’s Syndrome and Regional Differences in Disease Expression. In: Yazıcı, Y., Yazıcı, H. (eds) Behçet’s Syndrome. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-5641-5_3

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