Journal
IMMUNOLOGIC RESEARCH
Volume 61, Issue 1-2, Pages 53-62Publisher
HUMANA PRESS INC
DOI: 10.1007/s12026-014-8561-9
Keywords
Adult-onset Still's disease; Systemic-onset juvenile idiopathic arthritis; Autoinflammatory diseases; Reactive hemophagocytic lymphohistiocytosis; IL-1 beta; IL-18; Ferritin
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Funding
- Foundation for the Development of Internal Medicine in Europe (FDIME)
- Societe Nationale Francaise de Medecine Interne (SNFMI)
- Genzyme
- poste d'accueil'' at INSERM
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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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