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Review
. 2024:1448:573-582.
doi: 10.1007/978-3-031-59815-9_38.

Anti-Interferon-γ Therapy for Cytokine Storm Syndromes

Edward M Behrens  1 , Fabrizio de Benedetti  2
Affiliations
Review

Anti-Interferon-γ Therapy for Cytokine Storm Syndromes

Edward M Behrens et al. Adv Exp Med Biol. 2024.

Abstract

A vast body of evidence provides support to a central role of exaggerated production of interferon-γ (IFN-γ) in causing hypercytokinemia and signs and symptoms of hemophagocytic lymphohistiocytosis (HLH). In this chapter, we will describe briefly the roles of IFN-γ in innate and adaptive immunity and in host defense, summarize results from animal models of primary HLH and secondary HLH with particular emphasis on targeted therapeutic approaches, review data on biomarkers associated with activation of the IFN-γ pathway, and discuss initial efficacy and safety results of IFN-γ neutralization in humans.

Keywords: CXCL-9; Emapalumab; Infection-associated HLH; Interferon-γ; Interleukin-1; Interleukin-18; Interleukin-6; Macrophage activation syndrome; Mouse models; Primary HLH; Secondary HLH; Systemic juvenile idiopathic arthritis.

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References

    1. Ivashkiv LB. IFNgamma: Signalling, epigenetics and roles in immunity, metabolism, disease and cancer immunotherapy. Nat Rev Immunol. 2018;18(9):545–58. https://doi.org/10.1038/s41577-018-0029-z . - DOI - PubMed - PMC
    1. Schoenborn JR, Wilson CB. Regulation of interferon-gamma during innate and adaptive immune responses. Adv Immunol. 2007;96:41–101. https://doi.org/10.1016/S0065-2776(07)96002-2 . - DOI - PubMed
    1. Blouin CM, Lamaze C. Interferon gamma receptor: The beginning of the journey. Front Immunol. 2013;4:267. https://doi.org/10.3389/fimmu.2013.00267 . - DOI - PubMed - PMC
    1. de Weerd NA, Nguyen T. The interferons and their receptors–distribution and regulation. Immunol Cell Biol. 2012;90(5):483–91. https://doi.org/10.1038/icb.2012.9 . - DOI - PubMed - PMC
    1. Suarez-Ramirez JE, Tarrio ML, Kim K, Demers DA, Biron CA. CD8 T cells in innate immune responses: Using STAT4-dependent but antigen-independent pathways to gamma interferon during viral infection. MBio. 2014;5(5):e01978–14. https://doi.org/10.1128/mBio.01978-14 . - DOI - PubMed - PMC

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