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IL-1 Family Blockade in Cytokine Storm Syndromes

Randy Q Cron1

  • 1Children's of Alabama, Birmingham, AL, USA. randycron@uabmc.edu.

Advances in Experimental Medicine and Biology
|August 8, 2024
PubMed

Interleukin-1 is a prototypic proinflammatory cytokine that is elevated in cytokine storm syndromes (CSSs), such as secondary hemophagocytic lymphohistiocytosis (sHLH) and macrophage activation syndrome (MAS). IL-1 has many pleotropic and redundant roles in both innate and adaptive immune responses. Blockade of IL-1 with recombinant human interleukin-1 receptor antagonist has shown efficacy in treating CSS. Recently, an IL-1 family member, IL-18, has been demonstrated to be contributory to CSS in autoinflammatory conditions, such as in inflammasomopathies (e.g., NLRC4 mutations). Anecdotally, recombinant IL-18 binding protein can be of benefit in treating IL-18-driven CSS. Lastly, another IL-1 family member, IL-33, has been postulated to contribute to CSS in an animal model of disease. Targeting of IL-1 and related cytokines holds promise in treating a variety of CSS.

Keywords:
AnakinraCytokine storm syndromeIL-1 receptor antagonistIL-18 binding proteinInterleukin-1 (IL-1)Interleukin-1 receptor-like 1Interleukin-18 (IL-18)Interleukin-33 (IL-33)Macrophage activation syndromeSecondary hemophagocytic lymphohistiocytosis

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