孙娟 吴凤岐 鲁杰 何峰 刘哲伟.全身型幼年特发性关节炎与经典自身炎症疾病的比较[J].,2016,16(8):1584-1588 |
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 ClassicalAuto-inflammatory Disease |
|
DOI: |
中文关键词: 全身型幼年特发性关节炎 经典自身炎症性疾病 临床表现 发病机制 |
英文关键词: Systemic juvenile idiopathic arthritis Classic auto-inflammatory disease Manifestation Pathogenesis |
基金项目:首都卫生发展科研专项项目(2011-1008-02) |
|
摘要点击次数: 600 |
全文下载次数: 0 |
中文摘要: |
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。 |
英文摘要: |
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. |
查看全文
查看/发表评论 下载PDF阅读器 |
关闭 |