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

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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.

Conflict of interest

The authors declare that they have no conflict of interest.

Author information

Authors and Affiliations

  1. Department of Internal Medicine, Hopital de la Croix-Rousse, 103 grande rue de la Croix-Rousse, Lyon, 69004, France

    Yvan Jamilloux, Mathieu Gerfaud-Valentin & Pascal Sève

  2. University Claude Bernard-Lyon 1, Lyon, France

    Yvan Jamilloux, Mathieu Gerfaud-Valentin, Alexandre Belot, Thomas Henry & Pascal Sève

  3. Department of Biochemistry, University of Lausanne, Epalinges, Switzerland

    Yvan Jamilloux & Fabio Martinon

  4. International Research Center on Infectiology (CIRI), Inserm U1111, CNRS UMR5308-ENS de Lyon, Lyon, France

    Yvan Jamilloux & Thomas Henry

  5. Department of Paediatric Nephrology, Rheumatology, and Dermatology, Hôpital Femme-Mère-Enfant, Bron, 69500, France

    Alexandre Belot

Authors
  1. Yvan Jamilloux
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  2. Mathieu Gerfaud-Valentin
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  3. Fabio Martinon
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  4. Alexandre Belot
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  5. Thomas Henry
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  6. Pascal Sève
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Corresponding author

Correspondence to Yvan Jamilloux.

Additional information

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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  • DOI: https://doi.org/10.1007/s12026-014-8561-9

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