PMID- 35958573
OWN - NLM
STAT- MEDLINE
DCOM- 20220815
LR  - 20220818
IS  - 1664-3224 (Electronic)
IS  - 1664-3224 (Linking)
VI  - 13
DP  - 2022
TI  - Immune-mediated inflammatory diseases with chronic excess of serum 
      interleukin-18.
PG  - 930141
LID - 10.3389/fimmu.2022.930141 [doi]
LID - 930141
AB  - 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.
CI  - Copyright (c) 2022 Miyazawa and Wada.
FAU - Miyazawa, Hanae
AU  - Miyazawa H
AD  - Department of Pediatrics, School of Medicine, Institute of Medical, 
      Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan.
FAU - Wada, Taizo
AU  - Wada T
AD  - Department of Pediatrics, School of Medicine, Institute of Medical, 
      Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan.
LA  - eng
PT  - Journal Article
PT  - Review
DEP - 20220725
PL  - Switzerland
TA  - Front Immunol
JT  - Frontiers in immunology
JID - 101560960
RN  - 0 (Interleukin-18)
RN  - Lymphoproliferative Syndrome, X-Linked, 2
SB  - IM
MH  - Adult
MH  - *Arthritis, Juvenile
MH  - Genetic Diseases, X-Linked
MH  - Humans
MH  - Interleukin-18
MH  - *Lymphohistiocytosis, Hemophagocytic/etiology
MH  - Lymphoproliferative Disorders
MH  - *Macrophage Activation Syndrome
PMC - PMC9358977
OTO - NOTNLM
OT  - hemophagocytic lymphohistiocytosis
OT  - inflammasome
OT  - interleukin-18
OT  - macrophage activation syndrome
OT  - systemic autoinflammatory diseases
COIS- The authors declare that the research was conducted in the absence of any 
      commercial or financial relationships that could be construed as a potential 
      conflict of interest.
EDAT- 2022/08/13 06:00
MHDA- 2022/08/16 06:00
PMCR- 2022/01/01
CRDT- 2022/08/12 02:59
PHST- 2022/04/27 00:00 [received]
PHST- 2022/07/01 00:00 [accepted]
PHST- 2022/08/12 02:59 [entrez]
PHST- 2022/08/13 06:00 [pubmed]
PHST- 2022/08/16 06:00 [medline]
PHST- 2022/01/01 00:00 [pmc-release]
AID - 10.3389/fimmu.2022.930141 [doi]
PST - epublish
SO  - Front Immunol. 2022 Jul 25;13:930141. doi: 10.3389/fimmu.2022.930141. eCollection 
      2022.