儿童大动脉炎的诊断和治疗进展
Advances in diagnosis and treatment of Takayasu arteritis in children
大动脉炎是主动脉和肺动脉及其主要分支的慢性非特异性炎症,儿童患者的症状和体征常不典型,多为高血压、发热、关节炎和消瘦等非特异性表现.欧洲抗风湿病联盟和欧洲儿童风湿病学会提出了更适用于儿童的诊断标准,血管造影异常是诊断的必备条件,可以早期诊断并且监测疾病活动程度.其治疗除了传统的激素和免疫抑制剂以外,近年来一些新的免疫抑制剂以及生物制剂也取得了很好的疗效.
更多Takayasu arteritis (TA) is a devastating vasculitis of the aorta and its major branches,coronary and pulmonary arteries.The clinical manifestations in children are less specific than in adnlts:the disease in children presents with fever,arthralgias,vomiting,weight loss and hypertension.Conventional angiography,which is recognized as the golden standard in evaluating vascular lesions in TA,combined with computer tomography angiography (CTA),magnetic resonance angiography (MRA),ultrasonography,could not only provide important information for early diagnosis,but also detect disease activity.New immunosuppressive agents and biological therapies,such as TNF-a blocking agents,have been verified to be effective although corticosteroids and conventional immunosuppressive agents are still basic treatment.
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