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小儿 Takayasu 动脉炎:文献综述。

Pediatric Takayasu Arteritis: A Review of the Literature.

机构信息

Department of Pediatric Rheumatology, Istanbul University-Cerrahpasa, Cerrahpasa Medical School, Istanbul, Turkey.

出版信息

Curr Pediatr Rev. 2022;18(4):243-250. doi: 10.2174/1573396318666220304205518.

Abstract

Takayasu arteritis (TA) is the third most common vasculitis of childhood and is extremely rare. It is mainly characterized by chronic, autoimmune, and granulomatous inflammation of the aorta and its major branches. Women under 40 years of age are mostly affected. It occurs for the first time in childhood in about 30% of affected individuals. Initially, it presents nonspecific constitutional findings. Since there is no specific laboratory finding, diagnosis is challenging. The gold standard imaging method for diagnosis is conventional angiography. Delay in diagnosis can cause devastating consequences. Therefore, in cases presenting with nonspecific findings, with hypertension and high acute phase reactants, the diagnosis should be suspected and confirmed with appropriate imaging method, and treatment should be started immediately. Immunosuppressive agents are the mainstay of the treatment. Biological agents are successful in refractory cases, and endovascular revascularization methods are used in the treatment of complications.

摘要

Takayasu 动脉炎(TA)是儿童期第三常见的血管炎,极为罕见。它主要表现为主动脉及其主要分支的慢性、自身免疫性和肉芽肿性炎症。大多数受影响的人群为 40 岁以下的女性。约 30%的患者首次发生在儿童期。起初,它表现为非特异性全身表现。由于没有特定的实验室发现,诊断具有挑战性。诊断的金标准影像学方法是传统血管造影。诊断延迟可能导致灾难性后果。因此,对于出现非特异性表现、高血压和高急性期反应物的病例,应怀疑并通过适当的影像学方法确诊,并立即开始治疗。免疫抑制剂是治疗的主要方法。生物制剂对难治性病例有效,并且在治疗并发症时使用血管内血运重建方法。

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