Journal of Clinical Pediatrics >
Clinical characteristics of Behcet syndrome with cardiovascular aneurysms in children
Received date: 2025-08-20
Accepted date: 2025-11-26
Online published: 2026-02-02
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.
Key words: Behcet syndrome; aneurysm; cardiovascular involvement; child
LI Ming , YUE Tong , WEN Min , WU Feifei , ZHANG Dan , XU Yingjie , KANG Min , ZHU Jia , YAN Yuchun , LAI Jianming , WU Fengqi . Clinical characteristics of Behcet syndrome with cardiovascular aneurysms in children[J]. Journal of Clinical Pediatrics, 2026 , 44(2) : 146 -153 . DOI: 10.12372/jcp.2026.25e1015
| [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. |
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