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


Autoria(s): Jamilloux Y.; Gerfaud-Valentin M.; Martinon F.; Belot A.; Henry T.; Sève P.
Data(s)

2015

Resumo

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.

Identificador

http://serval.unil.ch/?id=serval:BIB_DAFB009FA0BE

isbn:1559-0755 (Electronic)

pmid:25388963

doi:10.1007/s12026-014-8561-9

isiid:000349004900009

Idioma(s)

en

Fonte

Immunologic Research, vol. 61, no. 1-2, pp. 53-62

Palavras-Chave #Adult-onset Still's disease; Systemic-onset juvenile idiopathic arthritis; Autoinflammatory diseases; Reactive hemophagocytic lymphohistiocytosis; IL-1 beta; IL-18; Ferritin
Tipo

info:eu-repo/semantics/article

article