Hyperinflammation: On the pathogenesis and treatment of macrophage activation syndrome.
HLH
inflammasomes
macrophage activation syndrome
pathogenesis
therapy
Journal
Acta paediatrica (Oslo, Norway : 1992)
ISSN: 1651-2227
Titre abrégé: Acta Paediatr
Pays: Norway
ID NLM: 9205968
Informations de publication
Date de publication:
Oct 2021
Oct 2021
Historique:
revised:
25
04
2021
received:
03
03
2021
accepted:
28
04
2021
pubmed:
3
5
2021
medline:
21
9
2021
entrez:
2
5
2021
Statut:
ppublish
Résumé
Macrophage activation syndrome (MAS) is a subtype of hemophagocytic lymphohistiocytosis (HLH) diseases. The underlying mechanism of these life-threatening disorders is impaired granule-mediated cytotoxicity exerted by natural killer (NK) cells and T lymphocytes. This function is meant for elimination of virus-infected cells, malignant cells and to prevent exaggerated immune responses. The normal outcome after an attack by NK or cytotoxic T cells is apoptosis of the target cell. This prevents cytotoxic inflammatory responses in adjacent tissues which occur after lytic cell death. Extensive cell lysis can even produce a cytokine storm, as evidenced in MAS. Programmed proinflammatory lytic cell death, pyroptosis, caused by activated inflammasomes is central in the pathogenesis of MAS. Pyroptosis mediates IL-18 cytokine release, which robustly stimulates NK and T cells to produce IFN-γ, the key macrophage-activating signal which initiates a burst of inflammatory cytokines and chemokines. Lytic cell death also mediates a discharge of the prototype alarmin high mobility group box protein 1 (HMGB1), a proinflammatory molecule present in all cells and that mediates the pathogenesis of MAS as outlined here. Therapeutic options to control causal factors operating in the pathogenesis of MAS are also discussed.
Substances chimiques
Cytokines
0
Types de publication
Journal Article
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
2717-2722Subventions
Organisme : Swedish Order of Freemasons in Stockholm
Informations de copyright
©2021 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.
Références
Ravelli A, Grom AA, Behrens EM, Cron RQ. Macrophage activation syndrome as part of systemic juvenile idiopathic arthritis: diagnosis, genetics, pathophysiology and treatment. Genes Immun. 2012;13(4):289-298.
Esteban YM, de Jong JLO, Tesher MS. An overview of hemophagocytic lymphohistiocytosis. Pediatr Ann. 2017;46(8):e309-e313.
Ramos-Casals M, Brito-Zeron P, Lopez-Guillermo A, Khamashta MA, Bosch X. Adult haemophagocytic syndrome. Lancet (London, England). 2014;383(9927):1503-1516.
Schulert GS, Canna SW. Convergent pathways of the hyperferritinemic syndromes. Int Immunol. 2018;30(5):195-203.
Crayne CB, Albeituni S, Nichols KE, Cron RQ. The immunology of macrophage activation syndrome. Front Immunol. 2019;10:119.
Halle S, Halle O, Förster R. Mechanisms and dynamics of T cell-mediated cytotoxicity in vivo. Trends Immunol. 2017;38(6):432-443.
Prager I, Watzl C. Mechanisms of natural killer cell-mediated cellular cytotoxicity. J Leukoc Biol. 2019;105(6):1319-1329.
Bloom BR, Bennett B. Mechanism of a reaction in vitro associated with delayed-type hypersensitivity. Science. 1966;153(3731):80-82.
Henter JI, Elinder G, Soder O, Hansson M, Andersson B, Andersson U. Hypercytokinemia in familial hemophagocytic lymphohistiocytosis. Blood. 1991;78(11):2918-2922.
Put K, Avau A, Brisse E, et al. Cytokines in systemic juvenile idiopathic arthritis and haemophagocytic lymphohistiocytosis: tipping the balance between interleukin-18 and interferon-γ. Rheumatology (Oxford, England). 2015;54(8):1507-1517.
Van Opdenbosch N, Lamkanfi M. Caspases in cell death, inflammation, and disease. Immunity. 2019;50(6):1352-1364.
Yasin S, Fall N, Brown RA, et al. IL-18 as a biomarker linking systemic juvenile idiopathic arthritis and macrophage activation syndrome. Rheumatology (Oxford, England). 2020;59(2):361-366.
Kaplanski G. Interleukin-18: biological properties and role in disease pathogenesis. Immunol Rev. 2018;281(1):138-153.
Dinarello CA, Novick D, Kim S, Kaplanski G. Interleukin-18 and IL-18 binding protein. Front Immunol. 2013;4:289.
Yang D, Han Z, Oppenheim JJ. Alarmins and immunity. Immunol Rev. 2017;280(1):41-56.
Kang R, Chen R, Zhang Q, et al. HMGB1 in health and disease. Mol Aspects Med. 2014;40:1-116.
Palmblad K, Schierbeck H, Sundberg E, et al. Therapeutic administration of etoposide coincides with reduced systemic HMGB1 levels in macrophage activation syndrome. Mol Med (Cambridge, Mass). 2021; (in press). https://doi.org/10.1186/s10020-021-00308-0
Yang H, Hreggvidsdottir HS, Palmblad K, et al. A critical cysteine is required for HMGB1 binding to Toll-like receptor 4 and activation of macrophage cytokine release. Proc Natl Acad Sci USA. 2010;107(26):11942-11947.
Andersson U, Yang H, Harris H. High-mobility group box 1 protein (HMGB1) operates as an alarmin outside as well as inside cells. Semin Immunol. 2018;38:40-48.
Deng M, Tang Y, Li W, et al. The endotoxin delivery protein HMGB1 mediates caspase-11-dependent lethality in sepsis. Immunity. 2018;49(4):740-753.e747.
Yang X, Cheng X, Tang Y, et al. Bacterial endotoxin activates the coagulation cascade through gasdermin D-dependent phosphatidylserine exposure. Immunity. 2019;51(6):983-996.e986.
Luedke CE, Cerami A. Interferon-gamma overcomes glucocorticoid suppression of cachectin/tumor necrosis factor biosynthesis by murine macrophages. J Clin Investig. 1990;86(4):1234-1240.
Faulds D, Goa KL, Cyclosporin BP. A review of its pharmacodynamic and pharmacokinetic properties, and therapeutic use in immunoregulatory disorders. Drugs. 1993;45(6):953-1040.
Canna SW, Cron RQ. Highways to hell: mechanism-based management of cytokine storm syndromes. J Allergy Clin Immunol. 2020;146(5):949-959.
Mehta P, Cron RQ, Hartwell J, Manson JJ, Tattersall RS. Silencing the cytokine storm: the use of intravenous anakinra in haemophagocytic lymphohistiocytosis or macrophage activation syndrome. Lancet Rheumatol. 2020;2(6):e358-e367.
Sen ES, Clarke SL, Ramanan AV. Macrophage Activation Syndrome. Indian J Pediatr. 2016;83(3):248-253.
Hande KR. Etoposide: four decades of development of a topoisomerase II inhibitor. Eur J Cancer. 1998;34(10):1514-1521.
Bergsten E, Horne A, Arico M, et al. Confirmed efficacy of etoposide and dexamethasone in HLH treatment: long-term results of the cooperative HLH-2004 study. Blood. 2017;130(25):2728-2738.
Horne A, von Bahr GT, Chiang SCC, et al. Efficacy of moderately dosed etoposide in macrophage activation syndrome - hemophagocytic lymphohistiocytosis (MAS-HLH). J Rheumatol. 2021; jrheum.200941. https://doi.org/10.3899/jrheum.200941
Chen GY, Tang J, Zheng P, Liu Y. CD24 and Siglec-10 selectively repress tissue damage-induced immune responses. Science. 2009;323(5922):1722-1725.
Tian RR, Zhang MX, Liu M, et al. CD24Fc protects against viral pneumonia in simian immunodeficiency virus-infected Chinese rhesus monkeys. Cell Mol Immunol. 2020;17(8):887-888.
Andersson U, Tracey KJ. Neural reflexes in inflammation and immunity. J Exp Med. 2012;209(6):1057-1068.
Koopman FA, Chavan SS, Miljko S, et al. Vagus nerve stimulation inhibits cytokine production and attenuates disease severity in rheumatoid arthritis. Proc Natl Acad Sci USA. 2016;113(29):8284-8289.
Huston JM, Gallowitsch-Puerta M, Ochani M, et al. Transcutaneous vagus nerve stimulation reduces serum high mobility group box 1 levels and improves survival in murine sepsis. Crit Care Med. 2007;35(12):2762-2768.
Hong GS, Zillekens A, Schneiker B, et al. Non-invasive transcutaneous auricular vagus nerve stimulation prevents postoperative ileus and endotoxemia in mice. Neurogastroenterol Motil. 2019;31(3):e13501.