Clinical features of vascular involvement in paediatric Behçet's syndrome: A retrospective, multi-center study

Abstract

Abstract Objective: Behçet’s syndrome (BS) can affect the vascular system, but little is known about the clinical manifestations of vasculo-BS (VBS) in children. The aim of this study was to explore the clinical characteristics of paediatric VBS. Methods: Clinical data of children with VBS treated at the Children's[A1] Hospital affiliated with the Capital Institute of Pediatrics and its sister hospitals from March 2013 to April 2023 were retrospectively analysed. Results: A total of 12 cases were identified, including 5 males and 7 females. Among them, 10 cases (83.3%) were treated at our department, accounting for 21.2% of the total BS cases in our department. The median age of onset was 9.5 years (range: 3-13 years). Vascular lesions were detected at the time of diagnosis in 10 cases, including 8 cases of pure arterial involvement ,2cases of pure venous involvementand and 2 cases of both arterial and venous involvement. Commonly affected arteries included the lower limb arteries (4/12), pulmonary artery, subclavian artery, renal artery, and superior mesenteric artery (3/12 each). Arterial wall thickening (6/12) and luminal stenosis (5/12) were common arterial lesions, with pulmonary artery thrombosis and arterial occlusion reported in 2 cases each. Venous wall thickening and luminal stenosis (2/12), as well as thrombosis (2/12), were common venous lesions, with superficial or deep venous thrombosis observed. Twelve cases received steroid therapy, 9 cases were treated using steroids combined with cyclophosphamide, and 8 cases were treated using steroids combined with thalidomide, while 9 cases using steroids combined with biologic agents. Surgical treatment was performed in 4 cases. Eight cases remained stable, 2 cases had recurrent activity, and 1 case died suddenly from cardiac causes. Conclusion: Paediatric VBS is rare, and vascular lesions are often identified concurrently with the diagnosis of BS. Arterial involvement is more common than venous involvement. Commonly affected arteries include the lower limb arteries, pulmonary artery, subclavian artery, renal artery, and superior mesenteric artery. Vascular lesions typically manifest as vascularwall thickening and/or luminal stenosis, with complications such as pulmonary artery embolism, arterial aneurysms, arterial occlusion, and venous thrombosis. Treatment with steroids combined with immunosuppressive agents and biologic agents is effective, but some cases still have a poor prognosis.