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Adult-onset Still's disease.
Journal article

Adult-onset Still's disease.

  • Gerfaud-Valentin M Hospices Civils de Lyon, Hôpital Universitaire de la Croix-Rousse, Service de médecine interne, F-69004 Lyon, France; Université Lyon I, F-69100 Villeurbanne, France; Université de Lyon, F-69000 Lyon, France.
  • Jamilloux Y Hospices Civils de Lyon, Hôpital Universitaire de la Croix-Rousse, Service de médecine interne, F-69004 Lyon, France; Inserm U1111, Centre International de Recherche en Infectiologie, F-69365 Lyon, France; Département de Biochimie, Université de Lausanne, 1006 Epalinges, Switzerland.
  • Iwaz J Université Lyon I, F-69100 Villeurbanne, France; Université de Lyon, F-69000 Lyon, France; Hospices Civils de Lyon, Service de Biostatistique, F-69000 Lyon, France; CNRS UMR 5558, Laboratoire de Biométrie et Biologie Evolutive, Equipe Biostatistique Santé, F-69310 Pierre-Bénite, France.
  • Sève P Hospices Civils de Lyon, Hôpital Universitaire de la Croix-Rousse, Service de médecine interne, F-69004 Lyon, France; Université Lyon I, F-69100 Villeurbanne, France; Université de Lyon, F-69000 Lyon, France. Electronic address: pascal.seve@chu-lyon.fr.
  • 2014-03-25
Published in:
  • Autoimmunity reviews. - 2014
Adult-onset Still's disease
Anakinra
Autoinflammatory disorder
Hemophagocytic lymphohistiocytosis
Hyperferritinemia
Macrophage activation syndrome
Adaptive Immunity
Adult
Genetic Predisposition to Disease
Humans
Immunity, Innate
Prognosis
Still's Disease, Adult-Onset
English First described in 1971, adult-onset Still's disease (AOSD) is a rare multisystemic disorder considered as a complex (multigenic) autoinflammatory syndrome. A genetic background would confer susceptibility to the development of autoinflammatory reactions to environmental triggers. Macrophage and neutrophil activation is a hallmark of AOSD which can lead to a reactive hemophagocytic lymphohistiocytosis. As in the latter disease, the cytotoxic function of natural killer cells is decreased in patients with active AOSD. IL-18 and IL-1β, two proinflammatory cytokines processed through the inflammasome machinery, are key factors in the pathogenesis of AOSD; they cause IL-6 and Th1 cytokine secretion as well as NK cell dysregulation leading to macrophage activation. The clinico-biological picture of AOSD usually includes high spiking fever with joint symptoms, evanescent skin rash, sore throat, striking neutrophilic leukocytosis, hyperferritinemia with collapsed glycosylated ferritin (<20%), and abnormal liver function tests. According to the clinical presentation of the disease at diagnosis, two AOSD phenotypes may be distinguished: i) a highly symptomatic, systemic and feverish one, which would evolve into a systemic (mono- or polycyclic) pattern; ii) a more indolent one with arthritis in the foreground and poor systemic symptomatology, which would evolve into a chronic articular pattern. Steroid- and methotrexate-refractory AOSD cases benefit now from recent insights into autoinflammatory disorders: anakinra seems to be an efficient, well tolerated, steroid-sparing treatment in systemic patterns; tocilizumab seems efficient in AOSD with active arthritis and systemic symptoms while TNFα-blockers could be interesting in chronic polyarticular refractory AOSD.
Language
  • English
Open access status
hybrid
Identifiers
Persistent URL
https://sonar.ch/global/documents/43151
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