全身型幼年特发性关节炎与经典自身炎症疾病的比较

Comparison of Systemic Juvenile Idiopathic Arthritis with Classical Auto-inflammatory Disease

幼年特发性关节炎(JIA)是一组病因不明、发病年龄小于16周岁、关节炎持续6周或6周以上疾病的统称,分为七种亚型。其中,全身型JIA的临床表现和发病机制与其他亚型明显不同,目前普遍认为其是一种自身炎症性疾病,非自身免疫性疾病。全身型JIA的临床表现与经典自身炎症性疾病具有很高的相似度,而二者的发病机制不仅有相似点,如促炎因子增加和相关信号通路活化,亦有不同点。自身炎症性疾病有明确与固有免疫系统相关的致病基因及家族史,而全身型JIA的致病基因目前尚无定论,也无明显家族遗传性,这是二者最大的区别。本文主要从临床症状特征、发病机制和家族史方面总结和比较了全身型幼年特发性关节炎与经典自身炎症疾病。

Juvenile idiopathic arthritis is a broad disease that defines as a clinically heterogeneous group of arthritis with unknown causes and begins before 16 years old. It encompasses several disease categories. Specially, systemic juvenile idiopathic arthritis（so-JIA） is very different from other subtypes in the terms of manifestation and pathogenesis. Most researchers classifies so-JIA into auto-inflammatory disease, rather than autoimmune disease. There exists highly similarity in the manifestation, On pathogenesis,there are both similarity and difference. The similarity reflects on the increase of pro-inflammatory cytokine and activation of related pathway. Auto-inflammatory diseases, not so-JIA, embody clear causing genes and family history. This review makes a comparison between so-JIA and classic auto-inflammatory disease in the terms of manifestation, pathogenesis and history.