Pathogenesis of adult-onset Still's disease: new insights from the juvenile counterpart
Pathogenesis of adult-onset Still's disease: new insights from the juvenile counterpart
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DOI：
10.1007/s12026-014-8561-9
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发表时间：
2015-02-01
影响因子：
4.4
通讯作者：
Seve, Pascal
中科院分区：
文献类型：
--
作者：
Jamilloux, Yvan;Gerfaud-Valentin, Mathieu;Seve, Pascal
成人型斯蒂尔病（AOSD）是一种罕见的炎症性疾病，其特征是典型的三联征：每日发热、关节炎和典型的鲑鱼色皮疹。最近积累的知识，主要是从遗传性自身炎症性疾病和全身性发作的幼年特发性关节炎（sJIA），提出了新的假设，AOSD的病理生理学。在本文中，我们首先讨论了AOSD和sJIA之间的连续体。然后，我们总结了目前关于潜在发病机制的假说：（1）感染假说;（2）自身炎症假说;（3）淋巴组织细胞假说;（4）高铁蛋白血症假说。最后，我们提出了最近的数据表明，AOSD患者分为两个不同的亚组与不同的课程，一个突出的全身功能和慢性关节炎。
Adult-onset Still's disease (AOSD) is a rare inflammatory disease characterized by the classical triad of daily fever, arthritis, and typical salmon-colored rash. Recent accumulation of knowledge, mostly arising from hereditary autoinflammatory diseases and from the systemic-onset juvenile idiopathic arthritis (sJIA), has given raise to new hypotheses on the pathophysiology of AOSD. In this review, we first discuss on the continuum between AOSD and sJIA. Then, we summarize current hypotheses on the underlying pathogenesis: (1) an infectious hypothesis; (2) an autoinflammatory hypothesis; (3) a lymphohistiocytic hypothesis; and (4) a hyperferritinemic hypothesis. Finally, we present the recent data suggesting that patients with AOSD fall into two distinct subgroups with different courses, one with prominent systemic features and one with chronic arthritis.