PMID- 37446301 OWN - NLM STAT- MEDLINE DCOM- 20230717 LR - 20230718 IS - 1422-0067 (Electronic) IS - 1422-0067 (Linking) VI - 24 IP - 13 DP - 2023 Jul 5 TI - IL-18 in Autoinflammatory Diseases: Focus on Adult Onset Still Disease and Macrophages Activation Syndrome. LID - 10.3390/ijms241311125 [doi] LID - 11125 AB - 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. FAU - Baggio, Chiara AU - Baggio C AD - Rheumatology Unit, Department of Medicine, University of Padova, 35128 Padova, Italy. FAU - Bindoli, Sara AU - Bindoli S AD - Rheumatology Unit, Department of Medicine, University of Padova, 35128 Padova, Italy. FAU - Guidea, Irina AU - Guidea I AD - Rheumatology Unit, Department of Medicine, University of Padova, 35128 Padova, Italy. FAU - Doria, Andrea AU - Doria A AUID- ORCID: 0000-0003-0548-4983 AD - Rheumatology Unit, Department of Medicine, University of Padova, 35128 Padova, Italy. FAU - Oliviero, Francesca AU - Oliviero F AUID- ORCID: 0000-0002-4958-5105 AD - Rheumatology Unit, Department of Medicine, University of Padova, 35128 Padova, Italy. FAU - Sfriso, Paolo AU - Sfriso P AUID- ORCID: 0000-0001-6432-7174 AD - Rheumatology Unit, Department of Medicine, University of Padova, 35128 Padova, Italy. LA - eng PT - Journal Article PT - Review DEP - 20230705 PL - Switzerland TA - Int J Mol Sci JT - International journal of molecular sciences JID - 101092791 RN - 0 (Interleukin-18) SB - IM MH - Adult MH - Humans MH - Interleukin-18/metabolism MH - *Still's Disease, Adult-Onset/diagnosis/drug therapy MH - *Macrophage Activation Syndrome/diagnosis MH - *Hereditary Autoinflammatory Diseases MH - Macrophages/metabolism PMC - PMC10342495 OTO - NOTNLM OT - adult-onset Still's disease OT - autoinflammatory diseases OT - interleukin (IL)-18 OT - macrophage activation syndrome COIS- A.D. received consultancy fees from GSK, Eli Lilly, AstraZeneca, and Otsuka. P.S. received grants from Novartis and Sobi. The other authors declare no conflict of interest. EDAT- 2023/07/14 13:08 MHDA- 2023/07/17 06:42 PMCR- 2023/07/05 CRDT- 2023/07/14 01:16 PHST- 2023/06/16 00:00 [received] PHST- 2023/06/30 00:00 [revised] PHST- 2023/07/03 00:00 [accepted] PHST- 2023/07/17 06:42 [medline] PHST- 2023/07/14 13:08 [pubmed] PHST- 2023/07/14 01:16 [entrez] PHST- 2023/07/05 00:00 [pmc-release] AID - ijms241311125 [pii] AID - ijms-24-11125 [pii] AID - 10.3390/ijms241311125 [doi] PST - epublish SO - Int J Mol Sci. 2023 Jul 5;24(13):11125. doi: 10.3390/ijms241311125.