Manifestations and predictors of vascular involvement in Behçet's disease

Behçet´s disease (BD) is a variable vessel vasculitis in which vascular involvement (VBD) occurs in up to 40% of patients and represents a poor prognosis marker. Predictors of VBD have been poorly defined.

We aimed to characterise VBD patients and to identify predictors of this clinical subtype.

We performed a single-centre, retrospective study including patients fulfilling the 2013 International Criteria for BD, registered in the Rheumatic Diseases Portuguese Registry (Reuma.pt) between October 2014 and May 2023. VBD was defined as arterial or venous manifestations attributable to BD and confirmed by imaging. Multivariable logistic regression was used to identify predictors of VBD.

A total of 157 BD patients were included, 24 (15%) of whom had vascular involvement. Seven (5%) presented VBD as the inaugural manifestation. The median age at VBD onset was 36.0 years, occurring a median of 8.9 years after BD onset. Venous involvement (20/24, 83%) predominated over arterial (10/24, 42%). Deep vein thrombosis (50%) and superficial thrombophlebitis (42%) were the most common venous events. Arterial manifestations included aneurysms (21%), pulmonary thrombosis (13%) and pseudoaneurysms (8%). Multiple-vessel involvement (54%) and recurrent events (58%) were common. On comparison with patients without VBD, male sex (OR 3.99, 95%CI 1.55-10.28) and ocular involvement at disease onset (OR 3.90, 95%CI 1.29-11.72) emerged as predictors of VBD.

In our BD cohort, vascular involvement occurred in 15% of patients, predominantly venous. Male sex and ocular involvement at disease onset were identified as predictors of VBD, suggesting their potential role as clinical markers for identifying high-risk patients.