Eleftheriou Despina, Brogan Paul A
Department of Paediatric Rheumatology, Institute of Child Health and Great Ormond St Hospital for Children, London, UK.
Best Pract Res Clin Rheumatol. 2009 Jun;23(3):309-23. doi: 10.1016/j.berh.2009.02.001.
Primary systemic vasculitides of the young are relatively rare diseases, but can have a significant morbidity and mortality. The purpose of this review is to provide an overview of the paediatric vasculitides. Vasculitides that predominantly affect children will be considered in more detail than vasculitic diseases that although are seen in children affect adults more commonly, such as the ANCA associated vasculitides. New classification criteria for childhood vasculitis have recently been proposed and are currently undergoing validation. Epidemiological clues continue to implicate infectious triggers in Kawasaki Disease and Henoch Schönlein purpura. Several genetic polymorphisms have now been described in the vasculitides that may be relevant in terms of disease predisposition or development of disease complications. Treatment regimens continue to improve, with the use of different immunosuppressive medications and newer therapeutic approaches such as biologic agents. However new challenges are looming in regards to the role of inflammation in endothelial health and the long term cardiovascular morbidity for children with primary systemic vasculitis. International multicenter collaboration is of utmost importance in order for us to further advance our understanding and improve the treatment and outcome of systemic vasculitis in the young.
儿童原发性系统性血管炎是相对罕见的疾病，但可导致显著的发病率和死亡率。本综述的目的是概述儿童血管炎。与主要影响儿童的血管炎相比，对虽可见于儿童但在成人中更常见的血管炎性疾病（如抗中性粒细胞胞浆抗体相关性血管炎）的讨论将相对简略。儿童血管炎的新分类标准最近已被提出，目前正在进行验证。流行病学线索继续表明，感染诱因与川崎病和过敏性紫癜有关。目前已在血管炎中描述了几种基因多态性，这可能与疾病易感性或疾病并发症的发生有关。随着不同免疫抑制药物的使用以及生物制剂等新治疗方法的出现，治疗方案不断改进。然而，关于炎症在血管内皮健康中的作用以及原发性系统性血管炎患儿的长期心血管发病率，新的挑战正在显现。国际多中心合作对于我们进一步加深理解、改善儿童系统性血管炎的治疗和预后至关重要。
