Li M, Sun X F, Xu Y J, Hou J, Zhang D, Chi Y, Zhu J, Kang M, Li S N, Su G X, Zhou Z X, Lai J M
Department of Rheumatism and Immunology, Children's Hospital, Capital Institute of Pediatrics, Beijing 100020, China.
Department of Radiology, Children's Hospital, Capital Institute of Pediatrics, Beijing 100020, China.
Zhonghua Er Ke Za Zhi. 2020 Dec 2;58(12):1001-1005. doi: 10.3760/cma.j.cn112140-20200719-00736.
To analyze the clinical characteristics of infantile Takayasu arteritis and the efficacy of infliximab (IFX). Clinical manifestations, laboratory investigations and infliximab intervention of a case with infantile Takayasu arteritis, who was admitted to Department of Rheumatism and Immunology, Children's Hospital, Capital Institute of Pediatrics in January 2018, were reviewed and analyzed. The related literature published from the beginning to March 2020 were retrieved from CNKI, Wanfang, SinoMed and PubMed with the keywords of"Takayasu arteritis","Infant" in both Chinese and English. This case was a 70-day-old boy admitted due to recurrent fever for 20 days. On admission, his blood pressure were 104/90, 95/59, 125/80, and 152/125 mmHg (1 mmHg=0.133 kPa) in the right arm, left arm, right leg, and left leg, respectively. The complete blood cell count showed leukocytosis (22.6×10/L), thrombocytosis (858×10/L) and mild anemia (80 g/L). He also had elevated erythrocyte sedimentation rate (119 mm/1h), serum ferritin (598 μg/L) and C-reactive protein (112 mg/L). Computed tomographic angiography (CTA) showed narrowing of the thoracic and abdominal aorta, with thickening and heterogenous enhancement of the vessel wall. Coronary artery ultrasound detected dilatation and wall thickening of the bilateral coronary arteries, and uneven dilatation of the middle segment of the right coronary artery, showing bead-like change. Vessel wall thickening was also found in the other main arteries, including both femoral arteries, axillary arteries, carotid arteries, and subclavian arteries, and both superficial femoral arteries were slightly narrowed in the distal segments. The diagnosis of TA was confirmed, and the boy was treated with infliximab monotherapy (5 mg/(kg·every time), a total of 13 times). Then his body temperature and all inflammatory markers were normalized, and the vascular pathology was resolved according to the radiography. No side effects such as allergy or infection were noted during the treatment. During the 2 years and 6 months of follow-up, the boy maintained normal growth and development. Literature review found 8 related articles, and one of them was in Chinese but had limited information. In the other 7 papers, a total of 7 infants with TA were reported. The most common symptom was fever (5 cases), and inflammatory markers usually elevated, and the most common affected artery was abdominal aorta (6 cases). Most cases were treated with glucocorticoid. TA is a rare disease in infants, usually presents with fever and increased inflammatory markers. At the early stage, infliximab monotherapy could effectively control the symptoms and ensure normal growth and development.
分析小儿大动脉炎的临床特征及英夫利昔单抗(IFX)的疗效。回顾并分析2018年1月首都儿科研究所附属儿童医院风湿免疫科收治的1例小儿大动脉炎患儿的临床表现、实验室检查及英夫利昔单抗干预情况。以“大动脉炎”“婴儿”的中英文为关键词,检索中国知网、万方、中国生物医学文献数据库及PubMed中2020年3月以前发表的相关文献。该病例为一名70日龄男婴,因反复发热20天入院。入院时,其右臂、左臂、右腿及左腿血压分别为104/90、95/59、125/80及152/125 mmHg(1 mmHg = 0.133 kPa)。血常规显示白细胞增多(22.6×10⁹/L)、血小板增多(858×10⁹/L)及轻度贫血(80 g/L)。红细胞沉降率(119 mm/1h)、血清铁蛋白(598 μg/L)及C反应蛋白(112 mg/L)也升高。计算机断层血管造影(CTA)显示胸主动脉和腹主动脉狭窄,血管壁增厚且强化不均匀。冠状动脉超声检查发现双侧冠状动脉扩张、管壁增厚,右冠状动脉中段不均匀扩张,呈串珠样改变。其他主要动脉,包括双侧股动脉、腋动脉、颈动脉及锁骨下动脉也发现血管壁增厚,双侧股浅动脉远端轻度狭窄。确诊为大动脉炎,给予英夫利昔单抗单药治疗(5 mg/(kg·次),共13次)。此后其体温及所有炎症指标恢复正常,影像学检查显示血管病变消退。治疗期间未发现过敏或感染等不良反应。在2年6个月的随访中,该患儿生长发育正常。文献复习共找到8篇相关文章,其中1篇为中文但信息有限。另外7篇论文共报道7例小儿大动脉炎患儿。最常见症状为发热(5例),炎症指标通常升高,最常受累动脉为腹主动脉(6例)。大多数病例采用糖皮质激素治疗。小儿大动脉炎是一种罕见疾病,通常表现为发热及炎症指标升高。早期采用英夫利昔单抗单药治疗可有效控制症状并确保正常生长发育。