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Immunologic Research
Published in: Immunologic Research 1-2/2015

01-02-2015 | CUTTING EDGE IN AUTOIMMUNITY

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

Authors: Yvan Jamilloux, Mathieu Gerfaud-Valentin, Fabio Martinon, Alexandre Belot, Thomas Henry, Pascal Sève

Published in: Immunologic Research | Issue 1-2/2015

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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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Metadata
Title
Pathogenesis of adult-onset Still’s disease: new insights from the juvenile counterpart
Authors
Yvan Jamilloux
Mathieu Gerfaud-Valentin
Fabio Martinon
Alexandre Belot
Thomas Henry
Pascal Sève
Publication date
01-02-2015
Publisher
Springer US
Published in
Immunologic Research / Issue 1-2/2015
Print ISSN: 0257-277X
Electronic ISSN: 1559-0755
DOI
https://doi.org/10.1007/s12026-014-8561-9

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