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Behçet's syndrome: disease manifestations, management, and advances in treatment

Abstract

The acne lesions characteristic of Behçet's syndrome are not sterile and are commonly observed in combination with arthritis. The two main nodular skin lesions—superficial thrombophlebitis and erythema nodosum—are equally frequent, and rather difficult to distinguish. Superficial thrombophlebitis is usually observed in combination with thrombosis in large veins, and thrombosis of the large veins usually clusters with dural sinus thrombi, which make up approximately 20% of all central nervous system (CNS) lesions of Behçet's syndrome. The remaining CNS lesions are parenchymal, mainly located in the brainstem, and associated with a graver prognosis than dural sinus thrombi. The presence of clinical clusters indicates that there are at least two pathogenetic pathways in Behçet's syndrome: a reactive arthritis pathway and a thrombophilia pathway. Research into the pathogenesis of Behçet's syndrome has shown that the most consistent genetic marker of Behçet's syndrome is HLA-B51; however, the genetic association of this true-to-form 'complex' disorder with HLA-B51 is only 20%, and a whole-genome study showed associations with 16 different loci. The severity of Behçet's syndrome and the mortality associated with it tend to decrease with time, and there is no associated increase in incidence of atherosclerosis. Although treatment of skin–mucosa manifestations, eye disease and pulmonary artery aneurysms has improved significantly in the past decades, the treatment of CNS lesions and thrombophilia are still problematic.

Key Points

  • Behçet's syndrome is characterized by articular, gastrointestinal, central nervous system and peripheral vascular manifestations

  • The highest incidence of Behçet's syndrome is in the Middle East, the Mediterranean basin and Far East regions

  • Behçet's syndrome does not fit in with the traditional autoimmune model–an 'autoinflammatory' mechanism has been proposed, and clinical symptom clusters, such as the association of acne with arthritis, might provide further insight

  • Standard treatments for eye disease and the mucocutaneous manifestations of Behçet's syndrome have improved significantly, although further improvements in the management of central nervous system diseases, thrombophilia and major vascular complications are warranted

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Yusuf Yazici, Gulen Hatemi, … Hasan Yazici

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Yazici, H., Fresko, I. & Yurdakul, S. Behçet's syndrome: disease manifestations, management, and advances in treatment. Nat Rev Rheumatol 3, 148–155 (2007). https://doi.org/10.1038/ncprheum0436

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