期刊
IMMUNOLOGIC RESEARCH
卷 61, 期 1-2, 页码 53-62出版社
HUMANA PRESS INC
DOI: 10.1007/s12026-014-8561-9
关键词
Adult-onset Still's disease; Systemic-onset juvenile idiopathic arthritis; Autoinflammatory diseases; Reactive hemophagocytic lymphohistiocytosis; IL-1 beta; IL-18; Ferritin
类别
资金
- Foundation for the Development of Internal Medicine in Europe (FDIME)
- Societe Nationale Francaise de Medecine Interne (SNFMI)
- Genzyme
- poste d'accueil'' at INSERM
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.
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