New insights into the management of Behçet syndrome

Behçet syndrome (BS) is a multisystem variable-vessel vasculitis characterized by recurrent mucocutaneous manifestations and severe organ involvement affecting the eyes, blood vessels, nervous system, and gastrointestinal tract. Although therapeutic advances over the past two decades have significantly improved outcomes, important clinical questions remain regarding optimal treatment selection, sequencing, durability, and long-term safety.

This narrative review summarizes current evidence on established and emerging therapies for BS, focusing on unresolved aspects of management. We discuss comparative data between infliximab and adalimumab, the evolving role of cyclophosphamide in major vascular involvement, treatment strategies in colchicine-resistant mucocutaneous and joint disease, the use of combination therapy with conventional immunosuppressives, and considerations for tapering or discontinuing biologics. We also review available evidence for alternative biologic and targeted therapies, including interleukin (IL)-1, IL-6, IL-12/23, IL-17 inhibitors, JAK inhibitors, and novel phosphodiesterase-4 inhibitors, as well as investigational agents. This review is based on a targeted literature search and the authors' expertise, including recent work for the updated 2025 EULAR recommendations for the management of BS.

Monoclonal anti-TNF antibodies remain the most supported biologic option across all disease manifestations. Emerging therapies show promise, but their long-term efficacy and safety require confirmation in controlled trials.