Clinical and diagnostic characteristics of arterial involvement in paediatric Behçet's disease

To explore the clinical features, diagnosis, treatment and prognosis of arterial involvement in paediatric Behçet's disease (BD) for clinical reference.

A retrospective cohort study analysed 76 paediatric BD patients (January 2013 - May 2024). Nineteen with arterial involvement were the experimental group, and 57 without vascular involvement were the control group.

The experimental group mainly involved medium-sized (15/19, 78.95%), large-sized (13/19, 68.42%) or both (9/19, 47.37%) arteries, most commonly abdominal aorta (8/19, 42.11%), pulmonary artery (7/19, 36.84%) and femoral artery (7/19, 36.84%). Notably, we found a high prevalence of coronary artery involvement, exclusively manifesting as left main coronary artery dilation. Lesions were mainly wall thickening (9/19, 47.37%), lumen dilation (8/19, 42.11%) and stenosis (7/19, 36.84%). Compared with the control group, it had later onset age (11.0 vs. 7.0 years), shorter disease duration (6.0 vs. 24.0 months), and higher incidences of fever, multi-organ involvement (neurological, renal, cardiac), and elevated inflammatory markers including C-reactive protein (CRP) and erythrocyte sedimentation rate (ESR). It also had more intensive treatment and surgery but had significantly higher rates of cerebrovascular accidents (15.79% vs. 0%), cardiac complications (31.58% vs. 0%), and mortality (10.53% vs. 0%).

Paediatric BD with arterial involvement is a severe phenotype with a poor prognosis, characterized by intense inflammation and multi-organ damage. This underscores the critical need for early identification, aggressive treatment, and close monitoring to improve long-term outcomes.