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Behçet Disease

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Periodic and Non-Periodic Fevers

Part of the book series: Rare Diseases of the Immune System ((RDIS))

Abstract

Behçet disease (BD) is a multisystemic autoinflammatory disorder of unknown etiology. BD is most common along the historical Silk Road from Far East Asia to the Mediterranean region. The disease is characterized by recurrent oral/genital ulcers and ocular, gastrointestinal, musculoskeletal, and nervous system involvement. BD is also a unique vasculitis affecting both arteries and veins of all sizes.

There are several sets of classification criteria for BD; however, the International Study Group (ISG) diagnostic/classification criteria and the recently proposed pediatric BD (PEDBD) criteria are the most widely used. There are no specific guidelines for the management of pediatric BD; however, very recently, the new European League Against Rheumatism (EULAR) recommendations for adult BD have been proposed.

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Authors and Affiliations

  1. Department of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey

    Erdal Sag, Yelda Bilginer & Seza Ozen

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  1. Erdal Sag
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  2. Yelda Bilginer
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  3. Seza Ozen
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  1. Department of Clinical Sciences and Community Health, University of Milano, Milano, Italy

    Rolando Cimaz

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Sag, E., Bilginer, Y., Ozen, S. (2020). Behçet Disease. In: Cimaz, R. (eds) Periodic and Non-Periodic Fevers. Rare Diseases of the Immune System. Springer, Cham. https://doi.org/10.1007/978-3-030-19055-2_9

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  • DOI: https://doi.org/10.1007/978-3-030-19055-2_9

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