{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,22]],"date-time":"2026-05-22T07:05:28Z","timestamp":1779433528963,"version":"3.53.1"},"reference-count":29,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,5,22]],"date-time":"2026-05-22T00:00:00Z","timestamp":1779408000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Vasculo-Beh\u00e7et\u2019s syndrome (VBS) is a rare subtype of Beh\u00e7et's syndrome (BS) characterized by vascular involvement, and its clinical profile in the pediatric population remains poorly characterized due to limited available data. This study aimed to describe the clinical characteristics, treatment strategies, and prognosis of pediatric VBS and to provide evidence for early identification and clinical management.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>A retrospective analysis was performed on the clinical data of 12 pediatric patients (\u226418 years old) with VBS admitted to three centers from January 2013 to December 2023. Demographic data, clinical manifestations, laboratory results, treatment regimens, and follow-up outcomes were collected and analyzed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Among the 12 patients, there were 5 boys and 7 girls, with a median age at onset of 9.5 years (range: 3\u201313 years). Arterial involvement was observed in 10\/12 cases, mainly characterized by vascular wall thickening (6\/12 cases) and luminal stenosis (5\/12 cases), involving the pulmonary artery, aorta, and multiple other sites. Venous involvement was found in 5\/12 cases, predominantly wall thickening with thrombosis (3\/12 cases). Multisystem involvement was common, including the skin (10\/12 cases), the gastrointestinal tract (9\/12 cases), and the urinary system (6\/12 cases). Inflammatory markers (CRP\/ESR) were elevated in 11\/12 cases. All patients received glucocorticoid therapy; 11\/12 cases received it combined with immunosuppressants, 9\/12 cases with biological agents, and 4\/12 cases underwent surgical treatment. With a median follow-up of 2 years (range: 4 months\u20135 years), 8\/12 cases achieved stable remission, 2\/12 cases (both complicated by aneurysms) had multiple relapses, 1\/12 case died of sudden cardiac death, and 1\/12 case showed no improvement.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Pediatric VBS is a rare and heterogeneous condition with frequent arterial involvement in this cohort. Vascular wall thickening may aid in the early recognition of this condition, while aneurysms may be associated with poorer outcomes.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3389\/fped.2026.1799746","type":"journal-article","created":{"date-parts":[[2026,5,22]],"date-time":"2026-05-22T06:12:18Z","timestamp":1779430338000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Clinical characteristics, treatment, and prognosis of pediatric Vasculo-Beh\u00e7et\u2019s syndrome: a retrospective analysis of 12 cases"],"prefix":"10.3389","volume":"14","author":[{"given":"Ming","family":"Li","sequence":"first","affiliation":[{"name":"Department of Rheumatology and Immunology, Capital Center for Children\u2019s Health, Capital Medical University, Capital Institute of Pediatrics","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xue","family":"Zhao","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, The Second Hospital of Hebei Medical University","place":["Shijiazhuang, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lian","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, General Hospital of Ningxia Medical University","place":["Yinchuan, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tong","family":"Yue","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Capital Center for Children\u2019s Health, Capital Medical University, Capital Institute of Pediatrics","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min","family":"Wen","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Capital Center for Children\u2019s Health, Capital Medical University, Capital Institute of Pediatrics","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fei-Fei","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Capital Center for Children\u2019s Health, Capital Medical University, Capital Institute of Pediatrics","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu-Fan","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Capital Center for Children\u2019s Health, Capital Medical University, Capital Institute of Pediatrics","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dan","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Capital Center for Children\u2019s Health, Capital Medical University, Capital Institute of Pediatrics","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ying-Jie","family":"Xu","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Capital Center for Children\u2019s Health, Capital Medical University, Capital Institute of Pediatrics","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jia","family":"Zhu","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Capital Center for Children\u2019s Health, Capital Medical University, Capital Institute of Pediatrics","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min","family":"Kang","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Capital Center for Children\u2019s Health, Capital Medical University, Capital Institute of Pediatrics","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu-Chun","family":"Yan","sequence":"additional","affiliation":[{"name":"Department of Radiology, Capital Center for Children\u2019s Health, Capital Medical University, Capital Institute of Pediatrics","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Feng-Qi","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Capital Center for Children\u2019s Health, Capital Medical University, Capital Institute of Pediatrics","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gai-Xiu","family":"Su","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Capital Center for Children\u2019s Health, Capital Medical University, Capital Institute of Pediatrics","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jian-Ming","family":"Lai","sequence":"additional","affiliation":[{"name":"Department of Rheumatology and Immunology, Capital Center for Children\u2019s Health, Capital Medical University, Capital Institute of Pediatrics","place":["Beijing, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,5,22]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"37","DOI":"10.1007\/s00296-018-4208-9","article-title":"Diagnostic\/classification criteria in pediatric Beh\u00e7et\u2019s disease","volume":"39","author":"Batu","year":"2019","journal-title":"Rheumatol Int"},{"key":"B2","doi-asserted-by":"publisher","first-page":"645","DOI":"10.1007\/s11739-018-1991-y","article-title":"Vascular Beh\u00e7et\u2019s syndrome: an update","volume":"14","author":"Emmi","year":"2019","journal-title":"Intern Emerg Med"},{"key":"B3","doi-asserted-by":"publisher","first-page":"127","DOI":"10.1007\/s00296-020-04777-0","article-title":"Performance of diagnostic criteria in pediatric Beh\u00e7et\u2019s disease","volume":"42","author":"Kurt","year":"2022","journal-title":"Rheumatol Int"},{"key":"B4","doi-asserted-by":"publisher","first-page":"111206","DOI":"10.1016\/j.ejrad.2023.111206","article-title":"Imaging characteristics and prognostic factors of Behcet\u2019s disease with arterial involvement: a long-term follow-up study","volume":"170","author":"Qian","year":"2024","journal-title":"Eur J Radiol"},{"key":"B5","doi-asserted-by":"publisher","first-page":"115","DOI":"10.1007\/s00296-021-05022-y","article-title":"Clinical presentation and treatment outcomes of arterial involvement in Beh\u00e7et\u2019s disease: a single-centre experience","volume":"42","author":"Chattopadhyay","year":"2022","journal-title":"Rheumatol Int"},{"key":"B6","doi-asserted-by":"publisher","first-page":"2018","DOI":"10.1093\/rheumatology\/keu233","article-title":"Vascular involvement in Beh\u00e7et\u2019s syndrome: a retrospective analysis of associations and the time course","volume":"53","author":"Tascilar","year":"2014","journal-title":"Rheumatology (Oxford)"},{"key":"B7","doi-asserted-by":"publisher","first-page":"232","DOI":"10.1016\/j.avsg.2006.12.004","article-title":"Major arterial involvement and review of Beh\u00e7et\u2019s disease","volume":"21","author":"Alpagut","year":"2007","journal-title":"Ann Vasc Surg"},{"key":"B8","doi-asserted-by":"publisher","first-page":"845","DOI":"10.1007\/s10067-013-2205-7","article-title":"Major vascular involvement in Beh\u00e7et\u2019s disease: a retrospective study of 796 patients","volume":"32","author":"Fei","year":"2013","journal-title":"Clin Rheumatol"},{"key":"B9","doi-asserted-by":"publisher","first-page":"801","DOI":"10.1007\/s12519-023-00785-9","article-title":"Clinical heterogeneity and five phenotypes identified in pediatric Beh\u00e7et\u2019s syndrome: a cohort study from Shanghai Beh\u00e7et\u2019s syndrome database","volume":"20","author":"Hu","year":"2024","journal-title":"World J Pediatr"},{"key":"B10","doi-asserted-by":"publisher","first-page":"3407","DOI":"10.1002\/ppul.25068","article-title":"Atypical cardiopulmonary manifestations in pediatric Beh\u00e7et\u2019s disease","volume":"55","author":"Ben-David","year":"2020","journal-title":"Pediatr Pulmonol"},{"key":"B11","doi-asserted-by":"publisher","first-page":"163","DOI":"10.1620\/tjem.255.163","article-title":"Vascular Behcet\u2019s disease preceded by fever of unknown origin: usefulness of ultrasonography for the detection of large-vessel vasculitis","volume":"255","author":"Matsumoto","year":"2021","journal-title":"Tohoku J Exp Med"},{"key":"B12","doi-asserted-by":"publisher","first-page":"283","DOI":"10.46497\/ArchRheumatol.2020.7459","article-title":"An adolescent presented with hemoptysis: pulmonary artery aneurysm in pediatric Beh\u00e7et\u2019s disease","volume":"35","author":"Ba\u015f Ikizo\u011flu","year":"2019","journal-title":"Arch Rheumatol"},{"key":"B13","doi-asserted-by":"publisher","first-page":"SI189","DOI":"10.1093\/rheumatology\/keac347","article-title":"Beh\u00e7et syndrome in children and adults: discovering similarities and differences by a comparative study","volume":"62","author":"Mastrolia","year":"2023","journal-title":"Rheumatology (Oxford)"},{"key":"B14","doi-asserted-by":"publisher","first-page":"248","DOI":"10.5606\/ArchRheumatol.2016.5749","article-title":"Cerebral venous thrombosis in Beh\u00e7et\u2019s disease patients compared to other causes of cerebral venous thrombosis: a retrospective study","volume":"31","author":"Sorgun","year":"2016","journal-title":"Arch Rheumatol"},{"key":"B15","doi-asserted-by":"publisher","first-page":"102060","DOI":"10.1016\/j.berh.2025.102060","article-title":"Update in imaging for large vessel vasculitis","volume":"39","author":"Nielsen","year":"2025","journal-title":"Best Pract Res Clin Rheumatol"},{"key":"B16","doi-asserted-by":"publisher","first-page":"654","DOI":"10.1007\/s00330-023-09994-2","article-title":"Normal pediatric values of carotid artery intima-media thickness measured by B-mode ultrasound and radiofrequency echo tracking respecting the consensus: a systematic review","volume":"34","author":"Moretti","year":"2024","journal-title":"Eur Radiol"},{"key":"B17","doi-asserted-by":"publisher","first-page":"1215","DOI":"10.3390\/life12081215","article-title":"Imaging of cerebral venous thrombosis","volume":"12","author":"Sadik","year":"2022","journal-title":"Life (Basel)"},{"key":"B18","doi-asserted-by":"publisher","first-page":"808","DOI":"10.1136\/annrheumdis-2018-213225","article-title":"2018 Update of the EULAR recommendations for the management of Beh\u00e7et\u2019s syndrome","volume":"77","author":"Hatemi","year":"2018","journal-title":"Ann Rheum Dis"},{"key":"B19","doi-asserted-by":"publisher","first-page":"801","DOI":"10.1148\/rg.243035091","article-title":"Thoracic manifestations of Beh\u00e7et disease at CT","volume":"24","author":"Hiller","year":"2004","journal-title":"Radiographics"},{"key":"B20","doi-asserted-by":"publisher","first-page":"62","DOI":"10.5152\/eurjrheum.2023.22043","article-title":"Beh\u00e7et\u2019s syndrome resembling Takayasu\u2019s arteritis with the distribution of arterial involvement: a case report and literature review","volume":"10","author":"Akkuzu","year":"2023","journal-title":"Eur J Rheumatol"},{"key":"B21","doi-asserted-by":"publisher","first-page":"61","DOI":"10.2177\/jsci.37.61","article-title":"An usual case of vasculo- and neuro-Beh\u00e7et\u2019s disease with MEFV mutations","volume":"37","author":"Okabe","year":"2014","journal-title":"Nihon Rinsho Meneki Gakkai Kaishi"},{"key":"B22","doi-asserted-by":"publisher","first-page":"bcr2018226039","DOI":"10.1136\/bcr-2018-226039","article-title":"Coeliac artery dissection as a rare manifestation of Beh\u00e7et\u2019s disease","volume":"2018","author":"Ocon","year":"2018","journal-title":"BMJ Case Rep"},{"key":"B23","first-page":"CD1","article-title":"Calculated decisions: Kawasaki disease diagnostic criteria","volume":"17","author":"Brown","year":"2020","journal-title":"Pediatr Emerg Med Pract"},{"key":"B24","doi-asserted-by":"publisher","first-page":"1769","DOI":"10.1007\/s10067-021-06031-9","article-title":"Clinical characteristics and radiographic outcomes of vascular Beh\u00e7et\u2019s disease involving the aorta or its major branches","volume":"41","author":"Lee","year":"2022","journal-title":"Clin Rheumatol"},{"key":"B25","doi-asserted-by":"publisher","first-page":"1238","DOI":"10.1093\/rheumatology\/keac452","article-title":"Pulmonary arterial wall thickness increased in Beh\u00e7et\u2019s disease patients with major organ involvement: is it a sign of severity?","volume":"62","author":"Kutlu\u011f A\u011fa\u00e7k\u0131ran","year":"2023","journal-title":"Rheumatology (Oxford)"},{"key":"B26","doi-asserted-by":"publisher","first-page":"152246","DOI":"10.1016\/j.semarthrit.2023.152246","article-title":"Diffuse generalized venulitis as the primary pathology of Beh\u00e7et\u2019s disease: a comprehensive magnetic resonance venography study","volume":"62","author":"Karadeniz","year":"2023","journal-title":"Semin Arthritis Rheum"},{"key":"B27","doi-asserted-by":"publisher","first-page":"109306","DOI":"10.1016\/j.clim.2023.109306","article-title":"Intima-media thickness of common femoral vein is increased in Beh\u00e7et\u2019s disease","volume":"250","author":"Sevik","year":"2023","journal-title":"Clin Immunol"},{"key":"B28","doi-asserted-by":"publisher","first-page":"9198506","DOI":"10.1155\/2019\/9198506","article-title":"The clinical features, risk factors, and outcome of aneurysmal lesions in Behcet\u2019s disease","volume":"2019","author":"Zhou","year":"2019","journal-title":"J Immunol Res"},{"key":"B29","doi-asserted-by":"publisher","first-page":"232","DOI":"10.1186\/s12887-019-1613-5","article-title":"Clinical manifestations and anti-TNF alpha therapy of juvenile Beh\u00e7et\u2019s disease in Taiwan","volume":"19","author":"Hu","year":"2019","journal-title":"BMC Pediatr"}],"container-title":["Frontiers in Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2026.1799746\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,5,22]],"date-time":"2026-05-22T06:12:19Z","timestamp":1779430339000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2026.1799746\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,5,22]]},"references-count":29,"alternative-id":["10.3389\/fped.2026.1799746"],"URL":"https:\/\/doi.org\/10.3389\/fped.2026.1799746","relation":{},"ISSN":["2296-2360"],"issn-type":[{"value":"2296-2360","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,5,22]]},"article-number":"1799746"}}