Données actualisées sur la physiopathologie, les phénotypes et les traitements de la maladie de Still de l'adulte [Pathophysiology, subtypes, and treatments of adult-onset Still's disease: An update].

Details

Serval ID
serval:BIB_DE4BEB75372A
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Données actualisées sur la physiopathologie, les phénotypes et les traitements de la maladie de Still de l'adulte [Pathophysiology, subtypes, and treatments of adult-onset Still's disease: An update].
Journal
La Revue de Médecine Interne
Author(s)
Gerfaud-Valentin M., Sève P., Hot A., Broussolle C., Jamilloux Y.
ISSN
1768-3122 (Electronic)
ISSN-L
0248-8663
Publication state
Published
Issued date
2015
Volume
36
Number
5
Pages
319-327
Language
french
Abstract
Adult-onset Still's disease is a rare and difficult to diagnose multisystemic disorder considered as a multigenic autoinflammatory syndrome. Its immunopathogenesis seems to be at the crossroads between inflammasomopathies and hemophagocytic lymphohistiocytosis, the most severe manifestation of the disease. According to recent insights in the pathophysiology and thanks to cohort studies and therapeutic trials, two phenotypes of adult-onset Still's disease may be distinguished: a systemic pattern, initially highly symptomatic and with a higher risk to exhibit life-threatening complications such as reactive hemophagocytic lymphohistiocytosis, where interleukin-1 blockade seems to be very effective, a chronic articular pattern, more indolent with arthritis in the foreground and less severe systemic manifestations, which would threat functional outcome and where interleukin-6 blockade seems to be more effective. This review focuses on these data.
Keywords
Adult-onset Still's disease, Interleukin-1, Interleukin-6, Autoinflammatory syndrome
Pubmed
Web of science
Open Access
Yes
Create date
29/06/2015 12:38
Last modification date
20/08/2019 16:02
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