2019
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Abstract: Objective. To assess the benefit of the recombinant human interleukin-1 receptor antagonist anakinra in treating pediatric patients with secondary hemophagocytic lymphohistiocytosis (HLH)/macrophage activation syndrome (MAS) associated with rheumatic and nonrheumatic conditions. Methods. A retrospective chart review of all anakinra-treated patients with secondary HLH/MAS was performed at Children's of Alabama from January 2008 through December 2016. Demographic, clinical, laboratory, and genetic characteristic… Show more

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“…A notably elevated ferritin value (e.g., > 700 ng/ml) should alert clinicians to additional diagnostic investigation so that therapeutic approaches can be considered without significant delay. As one might expect, the earlier the institution of treatment for CSS, the better the outcomes 11 .…”
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“…Prior to the use of anti-IFN-g treatment for CSS, other effective cytokine-targeted approaches were reported. Perhaps the best-studied is recombinant human IL-1 receptor antagonist (rhIL-1Ra, anakinra), which was recently reported to provide 73% survival among a cohort (n = 44) of rheumatic, infectious, and oncologic patients 11 . In a retrospective analysis of a prospective randomized and blinded placebo-controlled clinical trial, rhIL-1Ra also nearly doubled survival of sepsis patients with features of CSS 15 .…”
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“…Despite these interventions, mortality was as high as 20%. Identification of key mediators driving MAS-including IL-1β, IL-6, IL-18, and interferon-γ (IFNγ)-have inaugurated a new era of cytokine neutralization, potentially enabling a marked reduction in mortality (5,6). Herpes family viruses (e.g., Epstein-Barr virus) and influenza are major triggers of cytokine storm, both in systemic JIA and in patients without a preexisting immunologic diagnosis.…”
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“…Glucocorticoids remain a key first-line option, and clinical trials are urgently needed to test their efficacy and to identify optimal dosing, especially given the clear advantage of glucocorticoids in worldwide availability and cost. IL-1 blockade has shown particular promise as a treatment for cytokine storm syndrome, and high-dose regimens are safe even in the context of overt sepsis (5,10). Tocilizumab (anti-IL-6 receptor) is effective in cytokine release syndrome associated with CAR-T therapy, a syndrome notably reminiscent of COVID-19 in that many patients develop ARDS (11).…”
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“…IL-1β is one of the key inflammatory cytokines implicated in the cytokine storm syndrome 4,6,7 . Inhibition of IL-1βinduced cellular signaling with the IL-1 receptor (IL-1R) antagonist, Anakinra, has shown promise in treating cytokine storm and is proposed as a potential therapy for subjects with this severe complication of COVID-19 [8][9][10]11 . Yet, little is known about the immune cell subtypes most vulnerable to IL-1βinduced inflammatory signals in humans.…”
Section: Introductionmentioning