Clinical Rheumatology

, Volume 29, Issue 11, pp 1211–1216 | Cite as

Etiopathogenesis of Behçet's disease with emphasison the role of immunological aberrations

  • Violetta D. KapsimaliEmail author
  • Meletios A. Kanakis
  • George A. Vaiopoulos
  • Phaedon G. Kaklamanis
Review Article


Behçet's disease (BD) is a chronic multisystemic inflammatory disorder of unknown origin consisting of oral aphthous ulcers, ocular symptoms, skin lesions, and genital ulcerations. It has many features in common with systemic vasculitides and is more prevalent in countries along the ancient Silk route. Immune-mediated mechanisms play a major role in the pathogenesis of the disease, and inflammatory mediators are also involved. BD is not considered to be an autoimmune disorder, and the character of the disease needs to be clarified. Immunological aberrations in BD have been extensively studied by many investigators; genetic factors have been related to disease susceptibility, but their exact role in the development of disease is uncertain. Environmental factors such as infectious agents have also been implicated in the etiology of BD. However, the etiopathogenesis of the disease remains to be elucidated. Factors involved in the immunopathogenesis of BD with emphasis on the role of immunological aberrations are analyzed in this review.


Behçet's disease Genetic Immunologic Inflammatory mediators Pathophysiology Thrombophilic factors 





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Copyright information

© Clinical Rheumatology 2010

Authors and Affiliations

  • Violetta D. Kapsimali
    • 1
    Email author
  • Meletios A. Kanakis
    • 2
  • George A. Vaiopoulos
    • 2
  • Phaedon G. Kaklamanis
    • 3
  1. 1.Department of Microbiology, Medical SchoolUniversity of AthensAthensGreece
  2. 2.First Department of Internal MedicineUniversity of Athens, Laikon HospitalAthensGreece
  3. 3.Athens Medical CenterAthensGreece

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