儿童多发性大动脉炎17例临床特征分析

Clinical analysis of 17 patients with Takayasu arteritis in children

目的 探讨儿童多发性大动脉炎的临床特征及治疗转归情况,提高对该病的认识。方法 参考欧洲抗风湿病联盟-欧洲儿童风湿病学会-儿科风湿病国际研究组织(EULAR-PReS-PRINTO)诊断标准,回顾性分析重庆医科大学附属儿童医院2003年1月至2020年12月确诊为多发性大动脉炎患儿的临床资料。采用PVAS、ITAS及DEI.TaK量表评估疾病活动性。结果 17例中男5例、女12例,起病年龄1.3~14.0岁,确诊时病程18 d至6个月。常见临床表现为高血压12例(70.6%)、头晕头痛9例(52.9%)、血压差异7例(41.2%)、发热7例(41.2%)、血管杂音7例(41.2%)、呕吐6例(35.3%)、关节肿痛6例(35.3%)。炎症指标主要为红细胞沉降率(ESR)增快13例(84.6%),C反应蛋白升高11例(64.7%),白细胞升高11例(64.7%)。Numano分型中以Ⅴ型(6例,37.5%)及Ⅳ型(4例,25.0%)为主;腹主动脉受累最为常见(12例,70.6%),其次为左肾动脉(10例,58.8%);血管病变以管壁增厚(42支,45.7%)最为常见,其次为管腔狭窄(27支,29.3%)。病初PVAS与ITAS(r=0.789;P<0.05)和DEI.TaK(r=0.756;P<0.05)之间均存在强正相关。主要治疗方案为糖皮质激素及免疫抑制剂。4例因血压控制欠佳接受外科干预,其中球囊扩张术3例。随访半年至3年,8例好转,4例失访,3例恶化,2例死亡。结论 对于不明原因高血压合并急性期炎症指标升高者,需警惕多发性大动脉炎,应重视体格检查,加强疾病活动性评估。

Objective To explore the clinical characteristics and treatment outcome of child patients with Takayasu arteritis in order to improve the understancling of it.Methods A retrospective review was performed on all patients visiting our hospital from 2003 to 2020 who met EULAR-PReS-PRINTO classification criteria for childhood Takayasu arteritis.The Paediatric Vasculitis Activity Score(PVAS),Indian Takayasu Arteritis Activity Score(ITAS)and Disease Extent Index-Takayasu(DEI.Tak)were used to assess disease activity.Results The onset age of seventeen subjects(5 male and 12 female)was from 1.3 to 14 years old.The diagnostic delay was from 18 days to 6 months.The most common clinical manifestations were hypertension(12 cases, 70.6%),headache or dizziness(9 cases, 52.9%),blood pressure discrepancy(7 cases 41.2%),fever(7 cases 41.2%),bruits(7 cases 41.2%),vomit(6 cases, 35.3%)and arthralgia(6 cases, 35.3%).The inflammatory indexes were increased ESR in 13 cases(84.6%),increased CRP in 11 cases(64.7%) and increased WBC in 11 cases(64.7%).The most common angiographic involvement type according to the Numano classification system was type Ⅴ seen in six patients(37.5%),followed by type Ⅳ in four patients(25.0%).The abdominal aorta(12 cases, 70.6%)and left renal artery(10 cases 58.8%)were the most commonly involved vessels.The most common type of abnormality in our patients was thickening of vacular wall(42 cases, 45.7%)followed by stenosis(27,29.3%).At the beginning of disease, a medium or strong correlation was found between PVAS and ITAS(r=0.789;P<0.05)and between PVAS and DEI.TaK(r=0.756;P<0.05).Glucocorticoids and immunosuppressants were most commonly used medicine.Four patients required surgical intervention for hypertension with poor control in which there were 3 cases of balloon dilatation. The follow-up lasted from half a year to 3 years, and eight cases were improved, four cases were lost to follow up, three cases got worse and two cases died.Conclusion For patients with unexplained hypertension and high expression of inflammatory markers we should be alert to the occurrence of Takayasu arteritis, and we shoul pay more attention to physical examination and strengthen the assessment of disease activity.