Behçet's syndrome

Curr Opin Rheumatol. 1999 Jan;11(1):53-7. doi: 10.1097/00002281-199901000-00009.


There have not been any breakthroughs in understanding of the pathogenesis of Behçet's syndrome within the past year. We still do not know the meaning of the HLA-B51 association. The role of T cells in pathogenesis is still undefined, and it is not yet clear whether an extrinsic antigen or antigens produces the disease. Likewise the role of the neutrophil in pathogenesis is under scrutiny. More pediatric cases are being described. We are beginning to have a better understanding of central nervous system involvement, the prime pathology of which might not be vasculitis. Azathioprine, not a panacea, is nevertheless effective for long-term management. Thalidomide has clearly been shown to be effective in mucocutaneous disease even at a dose of 100 mg/d. However, relapses are seen with cessation of therapy.

Publication types

  • Review

MeSH terms

  • Azathioprine / therapeutic use
  • Behcet Syndrome / drug therapy
  • Behcet Syndrome / immunology
  • Behcet Syndrome / pathology*
  • HLA-B Antigens / immunology
  • HLA-B51 Antigen
  • Humans
  • Immunosuppressive Agents
  • Thalidomide / therapeutic use


  • HLA-B Antigens
  • HLA-B51 Antigen
  • Immunosuppressive Agents
  • Thalidomide
  • Azathioprine