Wargula Jennifer C
State University of New York at Buffalo, School of Medicine and Biomedical Sciences, USA.
Curr Opin Rheumatol. 2003 Sep;15(5):595-601. doi: 10.1097/00002281-200309000-00013.
Juvenile dermatomyositis is the most common of the idiopathic inflammatory myopathies in children. It is considered an autoimmune disease of relatively unknown etiology, although environmental exposures and infectious agents are thought to play a role in disease pathogenesis. More recently, data has become available regarding the molecular genetics of children affected with juvenile dermatomyositis and the impact these genes have on disease expression and clinical course. Additionally, features of the immune response, including specific pathways of the humoral and cellular immune systems, have been further described. This article summarizes the most recent advances in understanding the etiopathogenesis of juvenile dermatomyositis.
This article focuses on advances made in understanding the role that complement, soluble adhesion molecules, thrombospondin-1 levels, and genetics play in the evolution of juvenile dermatomyositis. It also describes microarray technology and gene expression profiling as means of identifying those genes overexpressed in affected children and thus likely involved in disease pathogenesis; microarray technology may also be used to distinguish dermatomyositis from the other inflammatory myopathies, as well as from other myopathies.
In better understanding the pathogenetic mechanisms whereby disease evolves and the means by which genetic profiles influence susceptibility to and expression of disease, immunotherapies to better treat juvenile dermatomyositis may become available in the future.
幼年皮肌炎是儿童特发性炎性肌病中最常见的一种。尽管环境暴露和感染因子被认为在疾病发病机制中起作用，但它被认为是一种病因相对不明的自身免疫性疾病。最近，有关患幼年皮肌炎儿童的分子遗传学以及这些基因对疾病表现和临床病程影响的数据已经可得。此外，免疫反应的特征，包括体液和细胞免疫系统的特定途径，也得到了进一步描述。本文总结了在理解幼年皮肌炎病因发病机制方面的最新进展。
本文重点关注在理解补体、可溶性黏附分子、血小板反应蛋白-1水平和遗传学在幼年皮肌炎发展过程中所起作用方面取得的进展。它还描述了微阵列技术和基因表达谱分析，作为识别在患病儿童中过度表达因而可能参与疾病发病机制的那些基因的手段；微阵列技术也可用于将皮肌炎与其他炎性肌病以及其他肌病区分开来。
在更好地理解疾病发展的致病机制以及基因谱影响疾病易感性和表现的方式方面，未来可能会有更好地治疗幼年皮肌炎的免疫疗法。
