论著

儿童白塞综合征伴心血管动脉瘤的临床特征分析

  • 李明 ,
  • 越桐 ,
  • 文旻 ,
  • 吴非霏 ,
  • 张丹 ,
  • 许瑛杰 ,
  • 康闽 ,
  • 朱佳 ,
  • 闫淯淳 ,
  • 赖建铭 ,
  • 吴凤岐
展开
  • 1.首都医科大学附属首都儿童医学中心 首都儿科研究所 风湿免疫科(北京 100020)
    2.首都医科大学附属首都儿童医学中心 首都儿科研究所 放射科(北京 100020)
赖建铭 电子信箱:laijm99@sina.com

收稿日期: 2025-08-20

  录用日期: 2025-11-26

  网络出版日期: 2026-02-02

基金资助

首都儿科研究所青年基金(QN-2024-18);首都儿科研究所青年基金(QN-2025-01);北京研究型病房卓越计划(BRWEP2024W102100100)

Clinical characteristics of Behcet syndrome with cardiovascular aneurysms in children

  • LI Ming ,
  • YUE Tong ,
  • WEN Min ,
  • WU Feifei ,
  • ZHANG Dan ,
  • XU Yingjie ,
  • KANG Min ,
  • ZHU Jia ,
  • YAN Yuchun ,
  • LAI Jianming ,
  • WU Fengqi
Expand
  • 1. Department of Rheumatology and Immunology, Capital Medical University, Capital Institute of Pediatrics, Beijing 100020, China
    2. Department of Radiology, Capital Center for Children's Health, Capital Medical University, Capital Institute of Pediatrics, Beijing 100020, China

Received date: 2025-08-20

  Accepted date: 2025-11-26

  Online published: 2026-02-02

摘要

目的 心血管动脉瘤是儿童白塞综合征(BS)极为罕见而严重的并发症,临床缺乏特异性且研究甚少。本研究旨在探讨儿童BS合并动脉瘤的临床特征及治疗策略。方法 回顾性分析2018年12月至2023年3月医院收治的3例BS合并动脉瘤患儿的临床资料。结果 同期共纳入36例儿童BS患者,其中3例发生动脉瘤(8.3%),男2例、女1例,BS起病年龄范围5~13岁,动脉瘤发现时间为BS起病后2个月~5年。2例肺动脉瘤伴原位血栓,1例主动脉右冠状动脉窦瘤;3例均合并其他血管病变(深静脉血栓、肺动脉血栓、主动脉夹层等)。发现动脉瘤时3例均发热、炎症指标升高,无胃肠道受累。3例均予激素联合免疫抑制剂(环磷酰胺、甲氨蝶呤)和生物制剂(肿瘤坏死因子单克隆抗体、托珠单抗)治疗,其中2例予手术干预(例1左下肺叶切除,例2 行Bentall手术)。例1、例3内科治疗后血管外症状稳定,肺动脉瘤无改善,例1、例2激素减量过程中疾病反复并出现新的脏器损害(例1累及神经系统,例2累及皮肤、心脏、肠道)。结论 儿童BS合并动脉瘤以肺动脉瘤最常见,常隐匿起病且多合并其他血管病变。推荐对炎症指标升高且无胃肠道受累的患儿定期行血管影像学筛查。治疗需个体化,可联合免疫抑制剂与手术,部分病例存在激素依赖。

本文引用格式

李明 , 越桐 , 文旻 , 吴非霏 , 张丹 , 许瑛杰 , 康闽 , 朱佳 , 闫淯淳 , 赖建铭 , 吴凤岐 . 儿童白塞综合征伴心血管动脉瘤的临床特征分析[J]. 临床儿科杂志, 2026 , 44(2) : 146 -153 . DOI: 10.12372/jcp.2026.25e1015

Abstract

Objective Cardiovascular aneurysm is an extremely rare and serious complication of Behcet syndrome (BS) in children, lacking clinical specificity and with very few studies. This study aims to explore the clinical characteristics and treatment strategies of BS complicated with aneurysms in children. Methods A retrospective analysis was conducted on the clinical data of 3 children with BS complicated with aneurysms who were admitted to hospital from December 2018 to March 2023. Results A total of 36 children with BS were included during the same period, among whom 3 patients (8.3%) developed aneurysms, including 2 boys and 1 girl. The age range of BS onset was 5 to 13 years old, and the time of aneurysm discovery was 2 months to 5 years after the onset of BS. Two cases involved pulmonary artery aneurysms (PAAs) with in-situ thrombosis, while one had an aneurysm at the right coronary sinus of the aorta. All patients presented with concurrent vascular pathologies (deep vein thrombosis, pulmonary arterial thrombosis, and aortic dissection). At aneurysm detection, all exhibited fever and elevated inflammatory markers, with no gastrointestinal involvement. All three patients were treated with corticosteroids combined with immunosuppressants (cyclophosphamide/methotrexate) and biologics (TNF-α inhibitors/tocilizumab), among whom two patients received surgical intervention (left lower lobectomy in Case 1 and Bentall procedure in Case 2). Following medical therapy, Cases 1 and 3 achieved extravascular stability, but PAAs showed no regression. During corticosteroid tapering, Cases 1 and 2 experienced recurrence with new organ damage (Case 1 involved the nervous system, and Case 2 involved the skin, heart, and intestines). Conclusions In children with BS, pulmonary artery aneurysms are the most common type of arterial aneurysm involvement. The condition often has an insidious onset and is frequently associated with other vascular lesions. Regular vascular imaging screening is recommended for children with elevated inflammatory markers and no gastrointestinal involvement. Treatment should be individualized, potentially combining immunosuppressive therapy and surgical intervention, as some cases exhibit steroid dependence.

参考文献

[1] Kul Cinar O, Romano M, Guzel F, et al. Paediatric Beh?et's disease: a comprehensive review with an emphasis on monogenic mimics[J]. J Clin Med, 2022, 11(5): 1278.
[2] Celik S, Yazici Y, Sut N, et al. Pulmonary artery aneurysms in Behcet's syndrome: a review of the literature with emphasis on geographical differences[J]. Clin Exp Rheumatol, 2015, 33(6Suppl 94): S54-S59.
[3] Saadoun D, Wechsler B, Desseaux K, et al. Mortality in Behcet's disease[J]. Arthritis Rheum, 2010, 62(9): 2806-2812.
[4] Batu ED. Diagnostic/classification criteria in pediatric Behcet's disease[J]. Rheumatol Int, 2019, 39(1): 37-46.
[5] 刘士远. 中华临床医学影像学胸部分册[M]. 北京: 北京大学医学出版社, 2015.
  Liu SY. Chinese clinical medical imaging: thoracic volume[M]. Beijing: Peking University Medical Press, 2015.
[6] Naik AL, Savlania A, Gupta A, et al. Isolated brachial artery aneurysm: a rare presentation of paediatric Behcet's disease[J]. Ann R Coll Surg Engl, 2021, 103(3): e94-e97.
[7] Zhuang LL, Liu HM, Li GM, et al. Dural sinus thrombosis and giant pulmonary artery aneurysm in paediatric Behcet's disease[J]. Clin Exp Rheumatol, 2020, 38(3): 558-566.
[8] Ben-David Y, Gur M, Ilivitzki A, et al. Atypical cardio-pulmonary manifestations in pediatric Behcet's disease[J]. Pediatr Pulmonol, 2020, 55 (12): 3407-3413.
[9] Is?yel E, Bakkalo?lu S, O?uz D, et al. An adolescent case of extensive Behcet`s disease successfully treated with Infliximab[J]. Turk J Pediatr, 2019, 61(4): 585-588.
[10] Kahn PJ, Yazici Y, Argilla M, et al. Asymptomatic giant coronary aneurysm in an adolescent with Behcet's syndrome[J]. Pediatr Rheumatol Online J, 2012, 10: 2.
[11] Ba? ?k?zo?lu N, Ata? E, Ergenekon AP, et al. An adolescent presented with hemoptysis: pulmonary artery aneurysm in pediatric Behcet's disease[J]. Arch Rheumatol, 2019, 35(2): 283-286.
[12] Li X, Fan X, Shen L, et al. Surgical management of a giant left distal pulmonary artery aneurysm caused by Behcet's disease[J]. Interact Cardiovasc Thorac Surg, 2020, 30(6): 943-944.
[13] Be?ba? N, Ozyürek E, Balkanci F, et al. Behcet's disease with severe arterial involvement in a child[J]. Clin Rheumatol, 2002, 21(2):176-179.
[14] Alada? ?iftdemir N, G?kalp S, Eren T. Is immuno-suppressive and thrombolytic therapy really effective in a patient with intracardiac thrombosis and pulmonary artery aneurysm due to Behcet's disease?[J]. Arch Rheumatol, 2019, 34(4): 451-456.
[15] Cook AL, Rouster-Stevens K, Williams DA, et al. Giant aneurysm of the left anterior descending coronary artery in a pediatric patient with Behcet's disease[J]. Pediatr Cardiol, 2010, 31(5): 700-702.
[16] Demircio?lu K?l?c B, Akbal?k Kara M, Ozcelik AA, et al. Brachial artery pseudoaneurysm: rare finding in childhood Behcet's disease[J]. Pediatr Int, 2017, 59(1): 109-110.
[17] Endo LM, Rowe SM, Romp RL, et al. Pulmonary aneurysms and intracardiac thrombi due to Beh?et's disease in an African-American adolescent with oculocutaneous albinism[J]. Clin Rheumatol, 2007, 26(9): 1537-1539.
[18] Ozen S, Bilginer Y, Besbas N, et al. Behcet disease: treatment of vascular involvement in children[J]. Eur J Pediatr, 2010, 169(4): 427-430.
[19] Vivante A, Bujanover Y, Jacobson J, et al. Intracardiac thrombus and pulmonary aneurysms in an adolescent with Behcet disease[J]. Rheumatol Int, 2009, 29(5): 575-577.
[20] 王树水, 张智伟, 曾少颖, 等. 儿童白塞病合并急性心肌梗死一例[J]. 中华风湿病学杂志, 2006, 10(6): 382-384.
  Wang SS, Zhang ZW, Zeng SY, et al. A case of Behcet's disease complicated with acute myocardial infarction in a child[J]. Zhonghua Fengshibingxue Zazhi, 2006, 10(6): 382-384.
[21] 卢晓倩, 王卓, 刘雷雷, 等. 儿童白塞病致椎动脉瘤一例[J]. 中华放射学杂志, 2017, 51(9): 719-720.
  Lu XQ, Wang Z, Liu LL, et al. A case of vertebral artery aneurysm caused by Behcet's disease in a child[J]. Zhonghua Fangshexue Zazhi, 2017, 51(9): 719-720.
[22] Zhou J, Shi J, Liu J, et al. The clinical features, risk factors, and outcome of aneurysmal lesions in Behcet's disease[J]. J Immunol Res, 2019, 2019: 9198506.
[23] Samreen I, Darji P, Genobaga S, et al. Pulmonary artery aneurysm in Behcet disease: medical, endovascular or surgical intervention[J]. Cureus, 2023, 15(11): e49368.
[24] Vallet H, Riviere S, Sanna A, et al. Efficacy of anti-TNF alpha in severe and/or refractory Behcet's disease: multicenter study of 124 patients[J]. J Autoimmun, 2015, 62: 67-74.
文章导航

/