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].

Détails

ID Serval
serval:BIB_DE4BEB75372A
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Institution
Titre
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].
Périodique
La Revue de Médecine Interne
Auteur(s)
Gerfaud-Valentin M., Sève P., Hot A., Broussolle C., Jamilloux Y.
ISSN
1768-3122 (Electronic)
ISSN-L
0248-8663
Statut éditorial
Publié
Date de publication
2015
Volume
36
Numéro
5
Pages
319-327
Langue
français
Résumé
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.
Mots-clé
Adult-onset Still's disease, Interleukin-1, Interleukin-6, Autoinflammatory syndrome
Pubmed
Web of science
Open Access
Oui
Création de la notice
29/06/2015 13:38
Dernière modification de la notice
20/08/2019 17:02
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