EULAR Updates Behçet’s Syndrome Management Recommendations

Overview

The European Alliance of Associations for Rheumatology (EULAR) has updated its management recommendations for Behçet’s syndrome, emphasizing earlier biologic use in patients with eye, vascular, and nervous system involvement. The update includes one new recommendation, seven modified recommendations, and four with wording changes, based on a comprehensive review of 81 studies.

Background

Behçet’s syndrome is a relapsing and remitting multisystem inflammatory disorder that can cause irreversible organ damage. Effective management requires individualized treatment and multidisciplinary care to maximize quality of life and prevent complications. The 2023 EULAR update revises the 2018 recommendations, incorporating new evidence from randomized controlled trials and observational studies. Key principles include early suppression of inflammation and shared decision-making with patients.

Data Highlights

Key Findings

• Earlier use of biologic DMARDs (bDMARDs) is recommended for eye, vascular, and nervous system involvement due to superior efficacy over conventional synthetic DMARDs.
• Colchicine remains first-line treatment for mucocutaneous and joint disease; apremilast or TNF-alpha inhibitors are options for colchicine-refractory cases.
• For Behçet’s uveitis, immunosuppressive therapy is mandatory; monoclonal anti-TNF-alpha antibodies, preferably infliximab, are recommended for sight-threatening posterior inflammation.
• High-dose glucocorticoids plus infliximab are now recommended for pulmonary and peripheral artery aneurysms, with cyclophosphamide as an alternative.
• Venous thrombosis management emphasizes glucocorticoids and immunosuppressives, with cautious use of anticoagulants and consideration of anti-TNF-alpha antibodies.
• For active parenchymal nervous system disease, first-line treatment includes high-dose glucocorticoids and immunosuppressives, preferably infliximab, reflecting a shift from previous azathioprine-first strategies.

Clinical Implications

Clinicians should consider earlier initiation of biologic therapies in patients with severe organ involvement to prevent irreversible damage. Colchicine remains foundational for mucocutaneous and joint manifestations, but escalation to biologics is supported for refractory disease. Multidisciplinary care and patient education remain essential components of management.

Conclusion

The updated EULAR recommendations provide evidence-based guidance emphasizing early and targeted biologic therapy in Behçet’s syndrome, aiming to improve outcomes and reduce organ damage. Ongoing research is needed to address treatment sequencing and management of refractory cases.

References

1. Hatemi et al. 2023 -- EULAR Recommendations for the Management of Behçet’s Syndrome