Zhu Jia, Kang Min, Xu Yingjie, Zhang Dan, Yue Tong, Li Ming, Wen Min, Wu Feifei, Su Gaixiu, Yang Yang, Niu Wenquan, Lai Jianming
Department of Rheumatology and Immunology, Capital Center for Children's Health, Capital Medical University, Capital Institute of Pediatrics, Beijing, 100020, China.
Department of Radiology, Capital Center for Children's Health, Capital Medical University, Capital Institute of Pediatrics, Beijing, 100020, China.
Pediatr Rheumatol Online J. 2025 Jun 10;23(1):64. doi: 10.1186/s12969-025-01111-5.
This study aims to summarize and explore the clinical characteristics, treatment strategies, and follow-up outcomes of infantile Takayasu arteritis (TA) in the Chinese Han population.
In this retrospective study, we collected clinical data, Kerr scores (the NIH score), and arterial involvement scores (AIS) of infants diagnosed with TA at the time of initial diagnosis and at 1, 3, 6, and 12 months following initial treatment. Prognostic differences between various treatment groups were also analyzed.
A total of 18 Chinese Han infants with TA were included. The average age of onset was 85.06 ± 54.64 days. The main clinical manifestations included fever (77.78%) and hypertension (55.56%). Both the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels were elevated above normal. The average Kerr score was 3.28 ± 0.67, and the AIS was 10.67 ± 4.30. The most commonly affected arteries were the coronary arteries (72.22%) and the abdominal aorta (72.22%), with arterial wall thickening present in all patients (100%). All patients were initially diagnosed with bacterial infections, and 16 were specifically diagnosed with Kawasaki disease empirically. Ten patients received infliximab treatment, and eight were treated with tocilizumab. At 6 months post-treatment, both the AIS and Kerr scores showed significant improvement compared to initial values (t = 2.469, = 0.019; t = 12.369, = 0.000). No deaths occurred during the 12-month follow-up. No significant differences were observed between the tocilizumab and infliximab treatment groups.
ITA is primarily characterized by fever and hypertension, along with elevated inflammatory biomarkers. The condition is easily diagnosed empirically with bacterial infections and Kawasaki disease, particularly in female infants around 3 months of age. Arterial involvement is often extensive and severe. Aggressive treatment within the first six months after diagnosis can significantly improve clinical outcomes. Both infliximab and tocilizumab demonstrated effective control of arterial inflammation.
本研究旨在总结并探讨中国汉族人群中婴儿型大动脉炎（TA）的临床特征、治疗策略及随访结果。
在这项回顾性研究中，我们收集了初次诊断时以及初次治疗后1、3、6和12个月诊断为TA的婴儿的临床数据、克尔评分（美国国立卫生研究院评分）和动脉受累评分（AIS）。还分析了不同治疗组之间的预后差异。
共纳入18例中国汉族TA婴儿。平均发病年龄为85.06±54.64天。主要临床表现包括发热（77.78%）和高血压（55.56%）。红细胞沉降率（ESR）和C反应蛋白（CRP）水平均高于正常。平均克尔评分为3.28±0.67，AIS为10.67±4.30。最常受累的动脉是冠状动脉（72.22%）和腹主动脉（72.22%），所有患者（100%）均存在动脉壁增厚。所有患者最初均被诊断为细菌感染，16例经验性诊断为川崎病。10例患者接受英夫利昔单抗治疗，8例接受托珠单抗治疗。治疗后6个月，AIS和克尔评分与初始值相比均有显著改善（t = 2.469，P = 0.019；t = 12.369，P = 0.000）。在12个月的随访期间无死亡发生。托珠单抗和英夫利昔单抗治疗组之间未观察到显著差异。
婴儿型TA主要表现为发热、高血压以及炎症生物标志物升高。该疾病易被经验性诊断为细菌感染和川崎病，尤其是在3个月左右的女婴中。动脉受累通常广泛且严重。诊断后前六个月积极治疗可显著改善临床结局。英夫利昔单抗和托珠单抗均能有效控制动脉炎症。
