Emerging advances in vascular Behçet syndrome

Vascular involvement is one of the main contributors to morbidity and mortality in Behçet syndrome. This review summarizes recent advances in the pathophysiology, imaging, and management of vascular Behçet syndrome.

Single-cell and translational studies support a central role for vessel-wall inflammation, neutrophil extracellular traps, and endothelial dysfunction. Common femoral vein wall ultrasonography, pulmonary artery wall assessment, and other vessel-wall imaging modalities suggest that vascular involvement may be more widespread than previously recognized. However, their diagnostic utility requires standardization and prospective validation across different cohorts and assessors. The updated 2025 EULAR recommendations position monoclonal anti-tumor necrosis factor alpha (TNFα) antibodies as first-line treatment options for both remission induction and maintenance across vascular manifestations. Anticoagulation is no longer restricted to refractory thrombosis, provided that bleeding-risk is assessed and pulmonary artery aneurysms are excluded.

Prompt immunosuppressive treatment with monoclonal anti-TNFα antibodies is now recommended as a first-line option for vascular involvement. Longitudinal cohorts and controlled trials are needed to validate vessel-wall imaging modalities.