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Immunologic Research

, Volume 61, Issue 1–2, pp 53–62 | Cite as

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

  • Yvan Jamilloux
  • Mathieu Gerfaud-Valentin
  • Fabio Martinon
  • Alexandre Belot
  • Thomas Henry
  • Pascal Sève
CUTTING EDGE IN AUTOIMMUNITY

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.

Keywords

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

Notes

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.

Conflict of interest

The authors declare that they have no conflict of interest.

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Copyright information

© Springer Science+Business Media New York 2014

Authors and Affiliations

  • Yvan Jamilloux
    • 1
    • 2
    • 3
    • 4
  • Mathieu Gerfaud-Valentin
    • 1
    • 2
  • Fabio Martinon
    • 3
  • Alexandre Belot
    • 2
    • 5
  • Thomas Henry
    • 2
    • 4
  • Pascal Sève
    • 1
    • 2
  1. 1.Department of Internal MedicineHopital de la Croix-RousseLyonFrance
  2. 2.University Claude Bernard-Lyon 1LyonFrance
  3. 3.Department of BiochemistryUniversity of LausanneEpalingesSwitzerland
  4. 4.International Research Center on Infectiology (CIRI), Inserm U1111, CNRS UMR5308-ENS de LyonLyonFrance
  5. 5.Department of Paediatric Nephrology, Rheumatology, and DermatologyHôpital Femme-Mère-EnfantBronFrance

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