• C Comarmond, B Wechsler, P Cacoub, and D Saadoun.
• Service de médecine interne et d'immunologie clinique, centre de référence des maladies autoimmunes rares, DHU i2B, inflammation, immunopathologie, biothérapie, université Pierre-et-Marie-Curie, groupe hospitalier Pitié-Salpêtrière, AP-HP, Paris 6, Paris, France.
• Rev Med Interne. 2014 Feb 1; 35 (2): 126-38.

AbstractBehçet's disease (BD) is a systemic large-vessel vasculitis characterized by a wide clinical spectrum including recurrent oral and genital ulcerations, uveitis, vascular, neurological, articular, and gastrointestinal manifestations. Therapeutic management of BD depends on the clinical presentation and organ involved. Although colchicine, non-steroidal anti-inflammatory agents and topical treatments with corticosteroids are often sufficient for mucocutaneous and joint involvement, a more aggressive approach with immunosuppressive agents is warranted for severe manifestations such as posterior uveitis, retinal vasculitis, vascular, neurological and gastrointestinal involvement. However, some patients still have refractory disease, relapses, sight threatening eye disease, or irreversible organ damage. Recent improvements in the understanding of the pathogenic mechanisms have led to the identification of potential targets and future therapies for BD. In contrast to current non-specific immunosuppressive agents, the emergence of immunomodulatory drugs provides the possibility of interfering with specific pathogenic pathways. Novel targeted immunosuppressive therapies might be used in the future for BD. Copyright © 2013 Société nationale française de médecine interne (SNFMI). Published by Elsevier SAS. All rights reserved.

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