IL-18 in Autoinflammatory Diseases: Focus on Adult Onset Still Disease and Macrophages Activation Syndrome

Int J Mol Sci. 2023 Jul 5;24(13):11125. doi: 10.3390/ijms241311125.


Interleukin-18 (IL-18) is a potent pro-inflammatory cytokine that is involved in various innate and adaptive immune processes related to infection, inflammation, and autoimmunity. Therefore, it is described as a key mediator of autoinflammatory diseases associated with the development of macrophage activation syndrome (MAS), including systemic juvenile idiopathic arthritis and adult-onset Still's disease. This review focuses on the role of IL-18 in inflammatory responses, placing emphasis on autoinflammatory diseases associated with chronic excess of serum IL-18, which correlate with clinical and biological signs of the disease. Therefore, it is useful for the diagnosis and monitoring of disease activity. Researchers are currently investigating IL-18's role as a therapeutic target for the treatment of inflammatory diseases. The inhibition of IL-18 signaling through recombinant human IL-18BP (IL-18 binding protein) seems to be an effective therapeutic strategy, though further studies are necessary to clarify its importance as a therapeutic target.

Keywords: adult-onset Still’s disease; autoinflammatory diseases; interleukin (IL)-18; macrophage activation syndrome.

Publication types

  • Review

MeSH terms

  • Adult
  • Hereditary Autoinflammatory Diseases*
  • Humans
  • Interleukin-18 / metabolism
  • Macrophage Activation Syndrome* / diagnosis
  • Macrophages / metabolism
  • Still's Disease, Adult-Onset* / diagnosis
  • Still's Disease, Adult-Onset* / drug therapy


  • Interleukin-18

Grants and funding

This research received no external funding.