26. Behçet's Syndrome

  1. Gary S. Hoffman MD, MS4,
  2. Cornelia M. Weyand MD, PhD5,
  3. Carol A. Langford MD, MHS4 and
  4. Jörg J. Goronzy MD, PhD5
  1. Yusuf Yazici MD1,
  2. Ismail Simsek MD2 and
  3. Hasan Yazici MD3

Published Online: 3 MAY 2012

DOI: 10.1002/9781118355244.ch26

Inflammatory Diseases of Blood Vessels, Second Edition

Inflammatory Diseases of Blood Vessels, Second Edition

How to Cite

Yazici, Y., Simsek, I. and Yazici, H. (2012) Behçet's Syndrome, in Inflammatory Diseases of Blood Vessels, Second Edition (eds G. S. Hoffman, C. M. Weyand, C. A. Langford and J. J. Goronzy), Wiley-Blackwell, Oxford, UK. doi: 10.1002/9781118355244.ch26

Editor Information

  1. 4

    Department of Rheumatic and Immunologic Diseases, Center for Vasculitis Care and Research, Cleveland Clinic, Lerner College of Medicine, Cleveland, OH, USA

  2. 5

    Department of Medicine, Stanford University School of Medicine, Stanford, CA, USA

Author Information

  1. 1

    New York University School of Medicine, NYU Hospital for Joint Diseases, New York, NY, USA

  2. 2

    Department of Medicine, Division of Rheumatology, Gülhane School of Medicine, Ankara, Turkey

  3. 3

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

Publication History

  1. Published Online: 3 MAY 2012
  2. Published Print: 8 JUN 2012

ISBN Information

Print ISBN: 9781444338225

Online ISBN: 9781118355244



  • Behcet's syndrome;
  • disease;
  • oral and genital ulcers;
  • pathergy reaction;
  • uveitis;
  • azathioprine;
  • HLA B51;
  • ethnic and geographic differences


Behçet's syndrome is a systemic vasculitis of unknown etiology involving the small and large vessels of both the venous and arterial systems. Distinct clusters of disease expression have been defined: the first includes superficial vein thrombosis, deep vein thrombosis and dural sinus thrombi; the second, acne, arthritis and enthesitis. The presence of these clusters suggests there might be more than one disease mechanism responsible for the disease manifestations in this complex disorder. European League Against Rheumatism (EULAR) guidelines are useful for the management of the disease in organ systems apart from the vascular, neurologic and gastrointestinal systems in which there is a lack of controlled studies.