Clinical characteristics, treatment, and prognosis of pediatric Vasculo-Behçet’s syndrome: a retrospective analysis of 12 cases

Objective Vasculo-Behçet’s syndrome (VBS) is a rare subtype of Behçet's syndrome (BS) characterized by vascular involvement, and its clinical profile in the pediatric population remains poorly characterized due to limited available data. This study aimed to describe the clinical characteristics, treatment strategies, and prognosis of pediatric VBS and to provide evidence for early identification and clinical management. Methods A retrospective analysis was performed on the clinical data of 12 pediatric patients (≤18 years old) with VBS admitted to three centers from January 2013 to December 2023. Demographic data, clinical manifestations, laboratory results, treatment regimens, and follow-up outcomes were collected and analyzed. Results Among the 12 patients, there were 5 boys and 7 girls, with a median age at onset of 9.5 years (range: 3–13 years). Arterial involvement was observed in 10/12 cases, mainly characterized by vascular wall thickening (6/12 cases) and luminal stenosis (5/12 cases), involving the pulmonary artery, aorta, and multiple other sites. Venous involvement was found in 5/12 cases, predominantly wall thickening with thrombosis (3/12 cases). Multisystem involvement was common, including the skin (10/12 cases), the gastrointestinal tract (9/12 cases), and the urinary system (6/12 cases). Inflammatory markers (CRP/ESR) were elevated in 11/12 cases. All patients received glucocorticoid therapy; 11/12 cases received it combined with immunosuppressants, 9/12 cases with biological agents, and 4/12 cases underwent surgical treatment. With a median follow-up of 2 years (range: 4 months–5 years), 8/12 cases achieved stable remission, 2/12 cases (both complicated by aneurysms) had multiple relapses, 1/12 case died of sudden cardiac death, and 1/12 case showed no improvement. Conclusion Pediatric VBS is a rare and heterogeneous condition with frequent arterial involvement in this cohort. Vascular wall thickening may aid in the early recognition of this condition, while aneurysms may be associated with poorer outcomes.