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

Abstract

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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Acknowledgments

Dr. Yvan Jamilloux acknowledges the Foundation for the Development of Internal Medicine in Europe (FDIME), the Société Nationale Française de Médecine Interne (SNFMI), and Genzyme for their help in funding his PhD research project. Dr. Yvan Jamilloux is supported by a “poste d’accueil” at INSERM.

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The authors declare that they have no conflict of interest.

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Correspondence to Yvan Jamilloux.

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Thomas Henry and Pascal Sève have contributed equally to this review.

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Jamilloux, Y., Gerfaud-Valentin, M., Martinon, F. et al. Pathogenesis of adult-onset Still’s disease: new insights from the juvenile counterpart. Immunol Res 61, 53–62 (2015). https://doi.org/10.1007/s12026-014-8561-9

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Keywords

  • Adult-onset Still’s disease
  • Systemic-onset juvenile idiopathic arthritis
  • Autoinflammatory diseases
  • Reactive hemophagocytic lymphohistiocytosis
  • IL-1β
  • IL-18
  • Ferritin