Comparative Study Sheds Light on Thrombosis Risk in Pediatric Behçet Syndrome

Results of a comparative study published in Seminars in Arthritis & Rheumatism uncovered several distinct clinical characteristics and management strategies for pediatric patients with Behçet syndrome and thrombosis, elucidating potential treatment approaches.

Behçet syndrome, marked by chronic inflammation impacting various bodily systems, presents a significant risk for mortality, primarily due to vascular complications. Despite thrombosis being a frequent complication, there remains a limited understanding of its full clinical spectrum, treatment strategies, and long-term consequences. Utilizing a European cohort of pediatric patients with Behçet syndrome, researchers compared the demographic and clinical features between those with vs without thrombosis.

A retrospective, multicenter, comparative study was conducted using data from the EUROFEVER registry. Patients diagnosed with Behçet syndrome before the age of 16 were included in the analysis. Individuals with vs without thrombosis were matched 1:2 based on sex, clinical variables, and follow-up time. Various imaging techniques were used to confirm the diagnosis of thrombosis.

The study included 37 pediatric patients with Behçet syndrome and thrombosis and 74 without thrombosis. The mean ages at Behçet syndrome onset were 12.02 and 12.85 years among those with vs without thrombosis, respectively; no differences were found between the sexes. Patients without vs with thrombosis were more often White (64.4% vs 45.9%), while Turkish patients more frequently had thrombosis (32.4% vs 27.4%).

Thrombosis occurred at disease onset among 21.6% of patients, while the overall median time to thrombosis was 11 months. Results of regression analyses showed that pustulosis (P =.001) and HLA-B51 positivity (P =.013) were linked to patients without thrombosis, while Turkish ethnicity (P <.001) was associated with thrombosis. Among patients with thrombosis, genital aphthosis (P =.011) was associated with Behçet syndrome onset while pustulosis (P =.030) was associated with the development of thrombosis.

Venous thrombosis (70.3%) was the most common type, particularly that involving the cerebral sinuses (43.8%).

Various prothrombotic factors, including lupus anticoagulant and anticardiolipin antibodies, were detected among patients with thrombosis. However, their individual roles in thrombosis development remain uncertain.

Immunomodulatory therapy was commonly initiated after thrombosis occurrence, increasing from 55.2% to 94.6% before and after. Anticoagulant (81.3%) and antiplatelet (21.2%) therapies were also frequently employed and appeared to be safe, with no reported bleeding complications.

Study limitations include the retrospective nature, potential selection bias, and restricted generalizability to patients outside of Europe.

The researchers concluded, “[P]ustulosis and fever appear to be more frequently concomitant to the development of thrombosis. Sinus veins were the most frequent thrombosis site.”

This article originally appeared on Rheumatology Advisor