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Review
. 2014 Dec 22:11:33-43.
doi: 10.2147/TCRM.S64951. eCollection 2015.

Treatment of adult-onset Still's disease: a review

Yvan Jamilloux  1 , Mathieu Gerfaud-Valentin  2 , Thomas Henry  3 , Pascal Sève  2
Affiliations
Review

Treatment of adult-onset Still's disease: a review

Yvan Jamilloux et al. Ther Clin Risk Manag. .

Abstract

Adult-onset Still's disease (AOSD) is a rare inflammatory disorder that has been recently classified as a polygenic autoinflammatory disorder. The former classification, based on the disease course, seems to be quite dated. Indeed, there is accumulating evidence that AOSD can be divided into two distinct phenotypes based on cytokine profile, clinical presentation, and outcome, ie, a "systemic" pattern and an "articular" pattern. The first part of this review deals with the treatments that are currently available for AOSD. We then present the different strategies based on the characteristics of the disease according to clinical presentation. To do so, we focus on the two subsets of the disease. Finally, we discuss the management of life-threatening complications of AOSD, along with the therapeutic options during pregnancy.

Keywords: adult-onset Still’s disease; anakinra; canakinumab; tocilizumab; treatment.

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Figures

Figure 1
Figure 1
Management algorithm for adult-onset Still’s disease. Abbreviations: AOSD, adult-onset Still’s disease; CS, corticosteroids; IVIg, intravenous immunoglobulin; NSAIDS, nonsteroidal anti-inflammatory drugs; RHL, reactive hemophagocytic lymphohistiocytosis.
Figure 2
Figure 2
Two subtypes of adult-onset Still’s disease. Abbreviations: AOSD, adult-onset Still’s disease; IFN, interferon; IL, interleukin; NK, natural killer; TNF, tumor necrosis factor.
See this image and copyright information in PMC

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