Abstract
ABSTRACTIntroduction: Adult-onset Still´s disease (AOSD) is a systemic inflammatory condition that affects mainly young people. The clinical course consists of two distinctive patterns: one with a predominance of systemic symptoms and another manifested by progressive chronic polyarthritis. Glucocorticoids remain the mainstay in the treatment of AOSD. However, biologic therapies are often required to achieve clinical remission and allow glucocorticoid discontinuation.Areas covered: The review summarizes the main retrospective and prospective studies, and case series on the use of the anti-interleukin (IL)-6 receptor tocilizumab in AOSD.Expert opinion: Since IL-6 serum levels are highly increased in both active systemic and polyarticular phenotypes, IL-6 blockade was considered to be a plausible therapeutic option for the management of AOSD. Tocilizumab, the only anti-IL-6-receptor antagonist currently available for AOSD, has proved to be effective for the management of refractory AOSD patients, including those with life-threatening complications. Nevertheless, there are some reports describing patients who are refractory to any therapy. Future research should focus on the identification of prognostic biomarkers that help us to tailor an individualized treatment for each type of patient and in the search of new disease activity indices that help us to monitor the response to the therapy more closely.
Highlights
Introduction tAdult-onset Stills disease (AOSD) is a systemic inflammatory disease of unknown ip origin affecting mainly young people with an estimated annual incidence between 0.16 r and 0.4/100,000 persons worldwide [1,2,3]
D extrapolation of the use of biologic therapies in AOSD was partially inferred from the te good results obtained in systemic onset juvenile idiopathic arthritis (sJIA). p Infectious and other environmental factors may trigger a systemic autoinflammatory e response in genetically predisposed individuals leading to a dysregulation of the c “inflammasome complex” with the overproduction of numerous pro-inflammatory Accytokines such as interleukin (IL)-1, interleukin 6 (IL-6), IL-18, interferon (IFN)-γ and tumor necrosis factor (TNF)-α [10,11]
AOSD is associated with a reduction in the glycosylated ip ferritin fraction, so that the combination of serum ferritin levels higher than 1000 μg/L cr with a glycosylated fraction
Summary
ISSN: 1471-2598 (Print) 1744-7682 (Online) Journal homepage: https://www.tandfonline.com/loi/iebt. N 4.5.8 Conclusions a Overall, and despite not being approved yet by the regulatory authorities, based on the M data shown in the different studies mentioned in this review, we conclude that TCZ is an effective drug for the overall management of AOSD, both for systemic and joint d manifestations, as well as for some severe complications of the disease. Further treatment options including sarilumab, a new anti-IL-6- agent with greater affinity for the IL-6 R than TCZ, inhibitors of intracellular signaling against Jak-1/Jak-2 and antagonists of IL-18 and IFN-γ need to be tested in patients with refractory AOSD In this line, an open-label, multicentre, dose-escalating phase II clinical trial on the safety and efficacy of tadekinig alpha, a recombinant human IL-18-binding protein (IL18BP), in AOSD has been recently published with promising results, the number of patients included was low [97,98]. Peer reviewers on this manuscript have no relevant financial relationships or otherwise Acc to disclose
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