Approaches to immunosuppression in Behçet's disease

Behçet's disease (BD) is a systemic large-vessel vasculitis characterized by a wide clinical spectrum including recurrent oral and genital ulcerations, uveitis, and vascular, neurological, articular, renal and gastrointestinal manifestations. Therapeutic management of BD depends on the clinical presentation and organ involved. Although colchicine, nonsteroidal 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 and vascular, neurological and gastrointestinal involvement. However, some patients still have refractory disease, relapse, 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 nonspecific 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.