Current management of Behçet's syndrome

Abstract

Behçet's syndrome (BS), or Behçet's disease (BD), is a multisystem inflammatory disorder that mainly affects population clusters along the Old Silk Road; however, it has been reported worldwide. The disease is currently classified as a vasculitis, characterized by sporadic outbreaks. The hallmarks of BS are recurrent, painful oral ulcers. Skin, eyes, central nervous system (CNS), gastrointestinal tract (GI) tract, and other organs may also be involved. CNS, GI, and major vascular involvement can be life‐threatening, and uveitis may lead to blindness. Immunosuppressive and immunomodulatory treatment is the mainstay of therapy for this inflammatory disease. Whereas the most frequently used agents for the treatment of BS have been corticosteroids, colchicine, and azathioprine, the introduction of anti‐tumor necrosis factor (TNF) agents and interferon‐α (IFN‐α) has begun to revolutionize the treatment of Behçet's eye disease and the management of other major organ manifestations. Data from randomized controlled trials (RCTs) showing beneficial effects for some of its disease manifestations are available for azathioprine, colchicine, cyclosporine, etanercept, IFN‐α depot‐methylprednisolone, and others. Currently, an evidence‐based approach to the management of BS is possible only for eye, mucocutaneous, and joint involvement, whereas recommendations for the treatment of gastrointestinal, neurological, and vascular disease remain based on expert opinion. Drug Dev Res 72:647–656, 2011. © 2011 Wiley Periodicals, Inc.