• Philippe Mertz, Véronique Hentgen, Guilaine Boursier, Ines Elhani, Laure Calas, Jerome Delon, and Sophie Georgin-Lavialle.
• Sorbonne université, hôpital Tenon, DMU3ID, APHP, ERN RITA, Paris, France; Centre de référence des maladies auto-inflammatoires et de l'amylose inflammatoire (CEREMAIA), Paris, France; Centre hospitalier de Versailles, 78150 le Chesnay, France; Institut Cochin, Inserm, CNRS, université Paris Cité, F-75014 Paris, France.
• Rev Med Interne. 2024 Aug 17.

AbstractAutoinflammatory diseases (AIDs) are conditions characterized by dysfunction of innate immunity, causing systemic inflammation and various clinical symptoms. The field of AIDs has expanded due to improved comprehension of pathophysiological mechanisms and advancements in genomics techniques. A new emerging category of AIDs is characterized by a significant increase in interleukin 18 (IL-18), a pro-inflammatory cytokine synthesized in macrophages and activated by caspase 1 via various inflammasomes. IL-18 plays a role in the regulation of innate and adaptive immunity. IL-18 is involved in various functions, such as the proliferation, survival, and differentiation of immune cells, tissue infiltration of immune cells, polarization of immune responses, and production of other pro-inflammatory cytokines. This review analyzes the literature on IL-18 regarding its functions and its implications in the diagnosis and treatment of AIDs. IL-18-associated AIDs comprise Still's disease and diseases associated with mutations in NLRC4, XIAP, CDC42, and PSTPIP1, as well as IL-18BP deficiencies. With the exception of PSTPIP1-associated diseases, these conditions all carry a risk of macrophagic activation syndrome. Measuring IL-18 levels in serum can aid in the diagnosis, prognosis, and monitoring of these diseases. Therapies targeting IL-18 and its signaling pathways are currently under investigation.Copyright © 2024 Société Nationale Française de Médecine Interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

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