Pathogenesis of adult-onset Still's disease: new insights from the juvenile counterpart

被引：151

作者：

Jamilloux, Yvan ^{[1
,2
,3
,4
]}

Gerfaud-Valentin, Mathieu ^{[1
,2
]}

Martinon, Fabio ^{[3
]}

Belot, Alexandre ^{[2
,5
]}

Henry, Thomas ^{[2
,4
]}

Seve, Pascal ^{[1
,2
]}

机构：

[1] Hop Croix Rousse, Dept Internal Med, F-69004 Lyon, France

[2] Univ Lyon 1, F-69365 Lyon, France

[3] Univ Lausanne, Dept Biochem, CH-1066 Epalinges, Switzerland

[4] CNRS UMR5308 ENS Lyon, Int Res Ctr Infectiol CIRI, Inserm U1111, Lyon, France

[5] Hop Femme Mere Enfant, Dept Paediat Nephrol Rheumatol & Dermatol, F-69500 Bron, France

来源：

IMMUNOLOGIC RESEARCH | 2015年 / 61卷 / 1-2期

关键词：

Adult-onset Still's disease; Systemic-onset juvenile idiopathic arthritis; Autoinflammatory diseases; Reactive hemophagocytic lymphohistiocytosis; IL-1; beta; IL-18; Ferritin; MACROPHAGE ACTIVATION SYNDROME; GENE-EXPRESSION PROFILES; IDIOPATHIC ARTHRITIS; CYTOKINE PROFILES; PROINFLAMMATORY CYTOKINE; RHEUMATOID-ARTHRITIS; CLINICAL-FEATURES; PERIPHERAL-BLOOD; INTERFERON-GAMMA; CELL-FUNCTION;

D O I：

10.1007/s12026-014-8561-9

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

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.

引用

页码：53 / 62

页数：10

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