儿童发病的大动脉炎15例临床分析

Clinical analysis of 15 children with Takayasu arteritis

目的研究儿童发病的大动脉炎患者的临床特点和治疗方案，提高对此病的认识，以求早期诊断，改善预后。方法收集我院1999年1月至2012年12月住院的大动脉炎患者的临床资料进行回顾性分析。结果15例患者中，男女比例为1：2，年龄14个月至15岁，平均（10±4）岁。按照EULAR／欧NJD童风湿病学协会（PRES）标准诊断大动脉炎。临床表现最为常见的是高血压13例（87％）、无脉或脉弱11例（73％）、心功能不全10例（67％）。接近213的患者在诊断时已经存在心功能不全的表现。临床分型最常见的为胸腹主动脉型9例（60％）。抗高血压，口服糖皮质激素和控制充血性心力衰竭是主要的治疗措施。3例结核菌素纯蛋白衍生物（PPD）试验阳性的患者接受了抗结核治疗，3例患者进行了血管旁路手术，1例患者进行了球囊动脉扩张术。结论大动脉炎威胁生命，且易进展，许多患者就诊时已处于疾病晚期，预后不佳，所以早期识别对于开展合理治疗是非常必要的。当患者出现无法解释的高血压或者炎症表现而没有明确的定位时，就应该考虑该病。

Objective To investigate the clinical features and treatment programs in children with Takayasu arteritis （TA） in order to improve awareness of the disease. Methods A retrospective study of hospi- talized children with TA in our hospital from Jan. 1999 to Dec. 2012 was performed. Results Between the 15 patients with TA, the ratio of male to female was 1：2. The onset was from 14 months to 15 years 01d, with average age at （10±4） years old. It is according to （the European League against Rheumatism/the Pediatric Rheumatology European Society （EULAR/PRES） criteria for the diagnosis of TA. The most common clinical manifestations are hypertension, which occurred in 13 cases（87%）, weak pulse or pulseless in 11 cases （73%）, and heart failure in 10 cases （67%）. About two-thirds of patients were diagnosed when the onset of heart failure occurred. The most common clinical type was type ]I, which occrred in 9 cases （60%）. Antihypertensive drugs, oral steroid and congestive heart failure controlling were the main treatment. Three patients with positive purifiedproteinderivative （PPD） test received anti-TB treatment. Three patients had vascular bypass surgery, one patient had percutaneous transluminal angioplasty. Conclusion TA may be life-threatening and progressive. Many patients are with advanced disease at the time of treatment, so the prognosis is generally poor. Early recognition is necessary to initiate appropriate therapy. The disease should be considered in patients with unexplained arterial hypertension or unexplained inflammatory syndromes without local signs.