Current management of Behçet syndrome

PURPOSE OF REVIEW: To summarize the most clinically relevant recent advances in the treatment of Behçet syndrome (BS), with emphasis on the updated 2025 EULAR recommendations and emerging evidence across different organ manifestations. RECENT FINDINGS: The updated EULAR recommendations support earlier use of monoclonal TNF inhibitors (TNFi), particularly in severe ocular, vascular, and neurologic involvement, and favor their continued use for maintenance in selected major-organ disease. Recent randomized and comparative studies provide further support for TNFi in Behçet uveitis and vascular involvement. Newer agents, including PDE-4 inhibitors and JAK inhibitors (JAKi), have shown promising efficacy in mucocutaneous, gastrointestinal, ocular, vascular, and neurologic disease, although most evidence for JAKi remains derived from small uncontrolled studies. Ustekinumab has also shown encouraging results in refractory intestinal BS. The role of anticoagulation in vascular BS remains uncertain. SUMMARY: Management of BS is increasingly organ- and risk-adapted, with earlier use of effective targeted therapies in patients at risk of irreversible damage. Despite encouraging results with several newer agents, comparative and high-quality evidence remains limited, highlighting the need for adequately powered studies with organ-specific outcomes.