Case report: Pediatric vascular Behçet's disease with prominent arterial involvement: a case series of five patients

Vascular Behçet's disease (VBD) rare and severe subtype of Behçet disease characterized by vascular inflammation. Its prevalence in the pediatric population is extremely low. This case series aims to delineate the clinical features and long-term outcomes of pediatric VBD to enhance clinical recognition and optimize management strategies.

We retrospectively reviewed five pediatric patients with VBD, all of whom were female. Arterial involvement was universal, with a subset of patients exhibiting concurrent venous disease. All patients showed a favorable response to glucocorticoids combined with immunosuppressants or biologic agents. Clinical manifestations were dominated by mucocutaneous and systemic features, including erythema nodosum, oral ulcers, genital ulcers, persistent fever, and abdominal pain. Laboratory evaluation revealed elevated inflammatory markers (C-reactive protein and/or erythrocyte sedimentation rate) in all cases. Vascular imaging confirmed the presence of vascular wall thickening and luminal stenosis. Colonoscopy at diagnosis identified multiple intestinal ulcers with surrounding mucosal hyperemia and edema in all five patients. "Clinical improvement" was defined as resolution of symptoms and normalized inflammatory markers; "resolution" meant complete disappearance of vascular lesions; "amelioration" meant ≥50% reduction in wall thickening or stenosis. During follow-up, all patients exhibited significant clinical improvement, with vascular lesions demonstrating resolution or significant amelioration on repeat imaging; intestinal ulcers healed in the four patients who underwent follow-up colonoscopy.

This pediatric case series highlights that VBD can affect vessels of various sizes and typically presents with a constellation of characteristic mucocutaneous and systemic symptoms. Early diagnosis and proactive vascular screening followed by combination immunosuppressive therapy were associated with favorable clinical and radiological outcomes. Causal conclusions cannot be drawn due to the lack of a control group. Increased awareness of this rare vasculopathy is critical for prompt intervention and improved patient prognosis.