| 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 |
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| 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) |
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| 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. |
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