Clinical characteristics of coronary artery involvement in children with Behcet's syndrome

To review the clinical features, treatment, and prognosis of coronary involvement in Behcet's syndrome in children.

Retrospectively analyzed medical records of BS patients admitted to our institution from 2012 to 2024.

Six children with BS with coronary involvement were admitted, including 2 males and 4 females. The mean age was 5.7 years and the mean course was 17.2 months. All of them had coronary dilatation, including 1 case with aneurysmal dilatation, 2 cases with bilateral coronary dilatation, 4 cases with unilateral dilatation, and 4 cases with valvular disease. No symptom for coronary artery dilation and all of coronary dilatation were detected by echocardiography. Manifestations of extracardiac vascular involvement included arteriovenous wall thickness and thrombosis. Five cases had only arterial involvement and one case had both artery and vein involvement. Pulmonary artery was involved in 1 case. All of them were treated with glucocorticoid and immunosuppressant, 1 child was treated with TNF-α, and 1 received aortic valvuloplasty due to a large number of aortic regurgitations. The patient with coronary aneurysm died suddenly after giving up treatment, and the other 4 cases were all improved to varying degrees.

Vascular involvement is an important factor affecting the prognosis of Behcet's syndrome. Patients with coronary involvement are occultic and difficult to diagnose. Both arteries and veins can be involved, and some patients have severe symptoms. Clinicians should attach great importance to early vascular screening, especially coronary artery. Early combined medication may help improve the prognosis. Key Points • Vascular involvement is an important factor affecting the prognosis of Behcet's syndrome. • Coronary involvement in BS is rare in children, which is predominantly coronary dilatation. • The onset of BS with coronary involvement is insidious and easy to be missed, so early coronary artery screening in children with BS should be emphasized. • Early combined medication may help improve the prognosis of BS with coronary involvement.