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Cited by 518

The DOI matches its Crossref record; 85 of the 85 references it cites were found in a registry or an index.

Adult-onset Still's disease

Open access
Gerfaud-Valentin, Mathieu, Jamilloux, Yvan, Iwaz, Jean, Sève, Pascal·2014·Autoimmunity Reviews

Abstract

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.

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Metadata

Authors
Gerfaud-Valentin, M., Jamilloux, Y., Iwaz, J., & Sève, P.
Venue
Autoimmunity Reviews
Publisher
Elsevier BV
Published
2014-07
Volume
13
Issue
7
Pages
708-722
ISSN
1568-9972 (ISSN-L)
OpenAlex
W2913532246
Cited by
518
References
85
FWCI
20.20
Type
journal article
Language
en
Open access
hybrid

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CoChat. (2026). Citation check: Adult-onset Still's disease. https://app.cochat.ai/papers/10.1016/j.autrev.2014.01.058
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Gerfaud-Valentin, M., Jamilloux, Y., Iwaz, J., & Sève, P. (2014). Adult-onset Still's disease. Autoimmunity Reviews. https://doi.org/10.1016/j.autrev.2014.01.058

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