儿童期起病的大动脉炎
Childhood-onset Takayasu Arteritis.
作者信息
Aeschlimann Florence A, Twilt Marinka, Yeung Rae S M
机构信息
Paediatric Immunology, Hematology and Rheumatology Unit, Hôpital Necker - Enfants Malades, Paris, France; Division of Paediatrics, Kantonsspital Winterthur, Winterthur, Switzerland.
Division of Rheumatology, Department of Paediatric, Alberta Children's Hospital, Alberta Children's Hospital Research Institute, Alberta, Canada; Department of Paediatrics, Cumming School of Medicine, University of Calgary, Calgary, Alberta, Canada.
出版信息
Eur J Rheumatol. 2020 Feb;7(Suppl1):S58-S66. doi: 10.5152/eurjrheum.2019.19195.
Childhood-onset Takayasu Arteritis (cTAK) is a rare, large-vessel type of vasculitis seen in children, mainly affecting the aorta and its major branches. Clinical manifestations are often severe and arise as a result of systemic and local inflammation, along with end-organ ischemia. Disease flares are common and the disease burden is high, with a significant rate of morbidity and mortality. Recent advances in understanding the underlying disease pathobiology resulted in the use of pathway-targeting agents, such as TNF- or IL-6 inhibitors with improved disease control. Nonetheless, the prognosis often remains guarded and the accrued damage is significant. This review aims at summarizing the recent evidence and observations regarding this condition, with a focus on pediatric publications.
儿童期发病的大动脉炎（cTAK）是一种罕见的累及大血管的血管炎，多见于儿童，主要影响主动脉及其主要分支。临床表现通常较为严重，是由全身和局部炎症以及终末器官缺血引起的。疾病发作很常见，疾病负担较重，发病率和死亡率较高。在理解潜在疾病病理生物学方面的最新进展导致了使用靶向通路药物，如肿瘤坏死因子或白细胞介素-6抑制剂，从而改善了疾病控制。尽管如此，预后往往仍然不佳，累积损害也很严重。本综述旨在总结关于这种疾病的最新证据和观察结果，重点关注儿科相关出版物。
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