Ting Tracy V, Hashkes Philip J
Section of Pediatric Rheumatology, Department of Rheumatic Diseases, Cleveland Clinic Foundation, Ohio 44195, USA.
Curr Opin Rheumatol. 2004 Sep;16(5):560-5. doi: 10.1097/01.bor.0000130287.62007.9f.
The purpose of this review is to provide an update on the new developments in pediatric vasculitis.
Because most childhood vasculitides are rare, few large systematic studies have been done. Studies of Henoch-Schönlein purpura have focused on pathogenesis and outcome. Genetic associations and molecular changes occurring during Henoch-Schönlein purpura, including cytokines, and endothelial and nitric oxide metabolism are discussed. Risk factors for renal involvement and poor renal outcome are described. Uncontrolled series of treatment protocols for severe Henoch-Schönlein purpura nephritis are mentioned. Several studies have focused on the pathogenesis of other primary vasculitides, especially polyarteritis nodosa. Series describing the clinical manifestations of childhood vasculitis and case reports of uncommon manifestations of vasculitis in children are presented. The efficacy of new therapies, including the use of thalidomide and biologic modifiers, has been shown in individual childhood cases; however, there are no controlled studies of these agents.
Besides studies of Henoch-Schönlein purpura, advances in pediatric vasculitis are few as a result of the rarity of most vasculitides in childhood. Multicenter collaboration is necessary to substantially increase the scientific base of investigating and treating childhood vasculitis.
本综述旨在提供小儿血管炎新进展的最新情况。
由于大多数儿童血管炎较为罕见，因此很少有大规模的系统性研究。对过敏性紫癜的研究集中在发病机制和预后方面。讨论了过敏性紫癜期间发生的遗传关联和分子变化，包括细胞因子、内皮细胞和一氧化氮代谢。描述了肾脏受累和不良肾脏预后的危险因素。提到了针对重症过敏性紫癜肾炎的未经验证的治疗方案系列。几项研究集中在其他原发性血管炎的发病机制上，尤其是结节性多动脉炎。介绍了描述儿童血管炎临床表现的系列研究以及儿童血管炎罕见表现的病例报告。在个别儿童病例中已显示了包括使用沙利度胺和生物调节剂在内的新疗法的疗效；然而，尚无对这些药物的对照研究。
除了对过敏性紫癜的研究外，由于大多数儿童血管炎的罕见性，小儿血管炎方面的进展很少。多中心合作对于大幅增加儿童血管炎调查和治疗的科学基础是必要的。
