Non-pulmonary arterial involvement in Behçet's disease: a multicenter retrospective case series

In Behçet's disease (BD), arterial involvement represents a significant clinical challenge associated with serious complications. The present study evaluates the characteristic features, affected vessels, lesion types, treatments, and outcomes in BD patients with nonpulmonary arterial involvement.

Included in this retrospective study were 65 BD patients whose interventions and clinical courses were accessed from medical records. The treatments administered included immunosuppressive (IS) therapies and surgical/endovascular procedures. Fisher's exact and t-test/Mann-Whitney U test were used for statistical analysis.

Fifty-nine of the patients (90.8%) were male. The median (Q1-Q3) follow-up was 4 (2-12.8) years. The most commonly affected vessels were the aorta (n = 21, 32.3%) and the femoral artery (n = 19, 29.2%). Aneurysm (n = 50, 63.2%) and thrombosis (n = 18, 22.7%) were the predominant lesions; 14 (21.5%) patients experienced arterial involvement while receiving IS therapy; and 11 (16.9%) had cardiac involvement. Cyclophosphamide and azathioprine were the most commonly used IS agents. Among the 39 (60%) patients who underwent surgical and/or interventional procedures, 32 (82%) had successful outcomes. A second arterial event was experienced by 15.3% of the sample during follow-up, and a third by 3%. Active disease was noted in nine (13.8%) patients at the time of their last visit, and of the six (9.2%) that died, most had both arterial and cardiac involvement.

Arterial involvement in BD mostly presents with aneurysms, especially in men. Surgical interventions in addition to IS therapies are required in approximately 50% of cases. As can be understood from the 15.3% relapse rate in the present study, arterial events are not uncommon, underlining the need for long-term monitoring and sustained IS treatment. Of the 9.2% of cases who died, most had both arterial and cardiac involvement.