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: Anakinra; Cytokine storm syndrome; IL-1 receptor antagonist; IL-18 binding protein; Interleukin-1 (IL-1); Interleukin-1 receptor-like 1; Interleukin-18 (IL-18); Interleukin-33 (IL-33); Macrophage activation syndrome; Secondary hemophagocytic lymphohistiocytosis.
© 2024. The Author(s), under exclusive license to Springer Nature Switzerland AG.