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Immune-mediated inflammatory diseases with chronic excess of serum interleukin-18
Review: Interleukin-18 (IL-18) is a proinflammatory cytokine that promotes various innate immune processes related to infection, inflammation, and autoimmunity. Patients with systemic juvenile idiopathic arthritis and adult-onset Still’s disease exhibit chronic excess of serum IL-18, which is associ...
Autores principales: | , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
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Frontiers Media S.A.
2022
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9358977/ https://www.ncbi.nlm.nih.gov/pubmed/35958573 http://dx.doi.org/10.3389/fimmu.2022.930141 |
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author | Miyazawa, Hanae Wada, Taizo |
author_facet | Miyazawa, Hanae Wada, Taizo |
author_sort | Miyazawa, Hanae |
collection | PubMed |
description | Review: Interleukin-18 (IL-18) is a proinflammatory cytokine that promotes various innate immune processes related to infection, inflammation, and autoimmunity. Patients with systemic juvenile idiopathic arthritis and adult-onset Still’s disease exhibit chronic excess of serum IL-18, which is associated with a high incidence of macrophage activation syndrome (MAS), although the mechanisms of IL-18 regulation in such diseases remain largely unknown. Similar elevation of serum IL-18 and susceptibility to MAS/hemophagocytic lymphohistiocytosis (HLH) have been reported in monogenic diseases such as X-linked inhibitor of apoptosis deficiency (i.e., X-linked lymphoproliferative syndrome type 2) and NLRC4-associated autoinflammatory disease. Recent advances in molecular and cellular biology allow the identification of other genetic defects such as defects in CDC42, PSTPIP1, and WDR1 that result in high serum IL-18 levels and hyperinflammation. Among these diseases, chronic excess of serum IL-18 appears to be linked with severe hyperinflammation and/or predisposition to MAS/HLH. In this review, we focus on recent findings in inflammatory diseases associated with and probably attributable to chronic excess of serum IL-18 and describe the clinical and therapeutical relevance of understanding the pathology of this group of diseases. |
format | Online Article Text |
id | pubmed-9358977 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2022 |
publisher | Frontiers Media S.A. |
record_format | MEDLINE/PubMed |
spelling | pubmed-93589772022-08-10 Immune-mediated inflammatory diseases with chronic excess of serum interleukin-18 Miyazawa, Hanae Wada, Taizo Front Immunol Immunology Review: Interleukin-18 (IL-18) is a proinflammatory cytokine that promotes various innate immune processes related to infection, inflammation, and autoimmunity. Patients with systemic juvenile idiopathic arthritis and adult-onset Still’s disease exhibit chronic excess of serum IL-18, which is associated with a high incidence of macrophage activation syndrome (MAS), although the mechanisms of IL-18 regulation in such diseases remain largely unknown. Similar elevation of serum IL-18 and susceptibility to MAS/hemophagocytic lymphohistiocytosis (HLH) have been reported in monogenic diseases such as X-linked inhibitor of apoptosis deficiency (i.e., X-linked lymphoproliferative syndrome type 2) and NLRC4-associated autoinflammatory disease. Recent advances in molecular and cellular biology allow the identification of other genetic defects such as defects in CDC42, PSTPIP1, and WDR1 that result in high serum IL-18 levels and hyperinflammation. Among these diseases, chronic excess of serum IL-18 appears to be linked with severe hyperinflammation and/or predisposition to MAS/HLH. In this review, we focus on recent findings in inflammatory diseases associated with and probably attributable to chronic excess of serum IL-18 and describe the clinical and therapeutical relevance of understanding the pathology of this group of diseases. Frontiers Media S.A. 2022-07-25 /pmc/articles/PMC9358977/ /pubmed/35958573 http://dx.doi.org/10.3389/fimmu.2022.930141 Text en Copyright © 2022 Miyazawa and Wada https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
spellingShingle | Immunology Miyazawa, Hanae Wada, Taizo Immune-mediated inflammatory diseases with chronic excess of serum interleukin-18 |
title | Immune-mediated inflammatory diseases with chronic excess of serum interleukin-18 |
title_full | Immune-mediated inflammatory diseases with chronic excess of serum interleukin-18 |
title_fullStr | Immune-mediated inflammatory diseases with chronic excess of serum interleukin-18 |
title_full_unstemmed | Immune-mediated inflammatory diseases with chronic excess of serum interleukin-18 |
title_short | Immune-mediated inflammatory diseases with chronic excess of serum interleukin-18 |
title_sort | immune-mediated inflammatory diseases with chronic excess of serum interleukin-18 |
topic | Immunology |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9358977/ https://www.ncbi.nlm.nih.gov/pubmed/35958573 http://dx.doi.org/10.3389/fimmu.2022.930141 |
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