Saulsbury F T
Division of Immunology and Rheumatology, Department of Pediatrics, University of Virginia Health System, Charlottesville, Virginia 22908, USA.
Curr Opin Rheumatol. 2001 Jan;13(1):35-40. doi: 10.1097/00002281-200101000-00006.
Although Henoch-Schönlein purpura (HSP) can occur at any age from infancy to adulthood, it is overwhelmingly a disease of childhood. Indeed, HSP is the most common vasculitis syndrome affecting children. The clinical features of HSP have been well documented, and the diagnosis is generally not difficult. However, there are substantial gaps in our understanding of the etiology, pathogenesis, and treatment of HSP. This article briefly reviews the clinical aspects of HSP and new information concerning therapy. The major focus of this review is recent information concerning abnormalities of immunoglobulin A1 glycosylation and the role of aberrantly glycosylated immunoglobulin A1 in the pathogenesis of HSP.
尽管过敏性紫癜(HSP)可发生于从婴儿期到成年期的任何年龄,但它绝大多数是一种儿童疾病。事实上,HSP是影响儿童的最常见血管炎综合征。HSP的临床特征已有充分记录,诊断通常并不困难。然而,我们对HSP的病因、发病机制和治疗的理解存在很大差距。本文简要回顾了HSP的临床方面以及有关治疗的新信息。本综述的主要重点是关于免疫球蛋白A1糖基化异常的最新信息以及异常糖基化的免疫球蛋白A1在HSP发病机制中的作用。