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目的探讨儿童多发性大动脉炎(TA)的临床特点及治疗转归,以提高对此病的认识。方法研究对象为1998年1月-2008年10月在北京大学第一医院儿科住院并确诊为TA的患儿10例,对其临床表现、实验室及影像学检查、治疗转归进行回顾性分析。结果10例TA患儿中男3例,女7例,男女比例为1 2.3;年龄4个月～9岁(平均5.5岁),平均确诊时间为7.6个月。最常见的临床症状为高血压(10例,100%),血管杂音(10例,100%),蛋白尿(7例,70%),抽搐(4例,40%)。临床分型Ⅱ型6例(占60%),Ⅲ型1例(占10%),Ⅳ型3例(占30%),以Ⅱ型胸腹主动脉型最常见,其中肾动脉受累90%。常规临床炎性活动指标如ESR、CRP、WBC升高均不明显。10例中结核感染6例均行抗结核治疗,6例行激素治疗,其中3例配合免疫抑制剂环磷酰胺、甲氨蝶呤治疗,同时予降压、抗凝等对症治疗。1例行肾动脉球囊扩张术加支架置入,1例行2次球囊动脉扩张术,1例行肾脏切除术,1例死于肾衰竭、心力衰竭、心源性休克。结论儿童TA临床表现不典型,急性炎性期多缺乏特异性表现。就诊时多处于疾病晚期(脏器缺血期),治疗效果不满意。应重视对结核感染的防控。对于多系统、多脏器损害的疾病,应全面检查,尤其是注重检查四肢血压、脉搏及血管杂音,注意TA的可能。早期进行血管彩超、CT、MRI等影像学检查可提高早期诊断率,提高疗效,改善预后。
Objective To investigate the clinical features and prognosis of children with multiple arteritis (TA) in order to improve their understanding of the disease. Methods From January 1998 to October 2008 in our hospital, Peking University First Hospital pediatric patients admitted to hospital and diagnosed with TA in 10 cases, the clinical manifestations, laboratory and imaging studies, the prognosis of treatment were retrospectively analyzed . Results There were 3 males and 7 females in 10 cases of TA with a male-female ratio of 1 2.3. The patients were 4 months to 9 years (mean 5.5 years) with an average diagnosis of 7.6 months. The most common clinical symptoms were hypertension (10 cases, 100%), vascular murmur (10 cases, 100%), proteinuria (7 cases, 70%) and convulsions (4 cases, 40%). There were 6 cases (60%) in type Ⅱ, 1 in type Ⅲ (10%) and 3 in type Ⅳ (30%), the most common type of thoracoabdominal aortic type Ⅱ, with renal artery involvement of 90% . Routine clinical inflammatory activity indicators such as ESR, CRP, WBC increased not obvious. In 10 cases, tuberculosis infection was performed in 6 cases and anti-tuberculosis treatment in 6 cases. Hormone treatment was performed in 6 cases. 3 cases were treated with cyclophosphamide and methotrexate, an immunosuppressive agent, and given symptomatic treatment such as hypotensive and anticoagulation. One patient underwent renal artery balloon dilatation plus stent implantation. One patient underwent balloon dilatation twice, one patient underwent nephrectomy, and one patient died of renal failure, heart failure and cardiogenic shock. Conclusion The clinical manifestations of TA in children are not typical, the acute phase of the lack of specific performance. Mostly in the treatment of advanced disease (organ ischemia), the treatment effect is not satisfied. Should pay attention to the prevention and control of tuberculosis infection. For multi-system, multiple organ damage, should be a comprehensive examination, in particular, focus on checking limbs blood pressure, pulse and vascular murmur, pay attention to the possibility of TA. Early vascular ultrasound, CT, MRI and other imaging studies can improve early diagnosis, improve efficacy and improve prognosis.