Behçet's disease is a multisystem vasculitis whose management is organ- and severity-based, combining classical immunosuppressants with targeted biologics for refractory or severe involvement. The 2025 EULAR update has nudged practice toward earlier use of biologics, particularly for sight- and organ-threatening disease.
Mucocutaneous and joint disease
Topical corticosteroids remain first-line for isolated oral and genital ulcers, and colchicine is first-line for arthritis and recurrent ulcers. For refractory mucocutaneous disease, apremilast (a PDE4 inhibitor) significantly reduced oral ulcers and improved quality of life versus placebo in the RELIEF trial and is now recommended for refractory cases; diarrhea, nausea, and headache are common. Azathioprine, methotrexate, and anti-TNF agents are options for more persistent disease.
Severe and major-organ involvement
For ocular disease, the 2025 recommendations mark a shift: monoclonal anti-TNF agents (preferably infliximab for sight-threatening inflammation) are now favored as first-line, with immunosuppression in all patients and an explicit caution against glucocorticoid monotherapy. Head-to-head data suggest adalimumab may achieve faster mucocutaneous responses than infliximab. For vascular, neurological, and gastrointestinal Behçet's, high-dose glucocorticoids are combined with immunosuppressants (cyclophosphamide or anti-TNF) for induction, and early anti-TNF is encouraged in organ- or life-threatening presentations. Interferon-α (effective for uveitis and mucocutaneous disease) and, in selected cases, tocilizumab or secukinumab serve as further options — though IL-17 inhibition warrants caution given reports of paradoxical disease.
| Organ system | First-line | Refractory / severe options |
|---|---|---|
| Oral/genital ulcers | Topical steroids, colchicine | Apremilast, azathioprine, anti-TNF, IFN-α |
| Joints | Colchicine | Immunosuppressants, anti-TNF, apremilast |
| Eye | Anti-TNF + immunosuppression (avoid steroid monotherapy) | IFN-α, tocilizumab |
| Vascular / neuro / GI | Steroids + immunosuppressant | Anti-TNF, cyclophosphamide, IFN-α |
Further reading: RELIEF apremilast (Hatemi et al., NEJM 2019); EULAR recommendations for the management of Behçet's syndrome, 2025 update; anti-TNF trials in Behçet's uveitis (2024).