Wang Xinning, Zhang Jinru, Wu Jing, Wang Xiaolei, Yao Xin, Niu Wenquan, Li Xiaohui, Li Jianguo
Department of Rheumatology and Immunology, Capital Institute of Pediatrics, Beijing, China.
Center of Evidence-Based Medicine, Capital Institute of Pediatrics, Beijing, China.
Clin Rheumatol. 2025 Jan;44(1):385-390. doi: 10.1007/s10067-024-07228-4. Epub 2024 Nov 25.
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.
回顾儿童白塞病冠状动脉受累的临床特征、治疗及预后。
回顾性分析2012年至2024年我院收治的白塞病患者的病历。
收治6例白塞病合并冠状动脉受累患儿，其中男2例，女4例。平均年龄5.7岁，平均病程17.2个月。均有冠状动脉扩张，其中动脉瘤样扩张1例，双侧冠状动脉扩张2例，单侧扩张4例，瓣膜病变4例。冠状动脉扩张均无症状，均经超声心动图检出。心外血管受累表现为动静脉壁增厚和血栓形成。5例仅有动脉受累，1例动静脉均受累。肺动脉受累1例。均采用糖皮质激素和免疫抑制剂治疗，1例患儿采用肿瘤坏死因子-α治疗，1例因大量主动脉反流接受主动脉瓣膜成形术。冠状动脉瘤患者放弃治疗后猝死，其余4例均有不同程度改善。
血管受累是影响白塞病预后的重要因素。冠状动脉受累患者隐匿，难以诊断。动静脉均可受累，部分患者症状严重。临床医生应高度重视早期血管筛查，尤其是冠状动脉。早期联合用药可能有助于改善预后。要点 • 血管受累是影响白塞病预后的重要因素。 • 儿童白塞病合并冠状动脉受累罕见，主要表现为冠状动脉扩张。 • 白塞病合并冠状动脉受累起病隐匿，易漏诊，应强调对白塞病患儿进行早期冠状动脉筛查。 • 早期联合用药可能有助于改善白塞病合并冠状动脉受累的预后。
