Macrophage activation syndrome in rheumatic disease: what is the role of the antigen presenting cell?
- PMID: 18295734
- DOI: 10.1016/j.autrev.2007.11.025
Macrophage activation syndrome in rheumatic disease: what is the role of the antigen presenting cell?
Abstract
Macrophage Activation Syndrome (MAS), alternatively referred to as secondary hemophagocytic lymphohistiocytosis (HLH), is a complication of many rheumatic diseases, most commonly Systemic Juvenile Idiopathic Arthritis (SJIA). MAS consists of a fulminant picture of pan-cytopenia, hectic high fevers, hepatosplenomegaly, lymphadenopathy, rash, and central nervous systemic inflammation. It can arise from genetic defects in the cytotoxic effector response of CD8+ T-cells, resulting in an inability to terminate antigen presentation, which in turn leads to uncontrolled immune activation. However, in the case of most rheumatic diseases, no such defect in cytotoxic killing is present. Little is known about what the contributions from the antigen presenting cells are in the pathogenesis of MAS. In fact, macrophages may be playing a regulatory, anti-inflammatory in MAS. We review the proposed pathogenesis of MAS/HLH, what role macrophages may play in the disease, and the relationship of MAS to its most common associated rheumatic disease, SJIA.
Similar articles
-
Macrophage activation syndrome: a frequent but under-diagnosed complication associated with rheumatic diseases.Med Sci Monit. 2008 Mar;14(3):RA27-36. Med Sci Monit. 2008. PMID: 18301366 Review.
-
Mutations of the hemophagocytic lymphohistiocytosis-associated gene UNC13D in a patient with systemic juvenile idiopathic arthritis.Arthritis Rheum. 2008 Feb;58(2):567-70. doi: 10.1002/art.23199. Arthritis Rheum. 2008. PMID: 18240215
-
Occult macrophage activation syndrome in patients with systemic juvenile idiopathic arthritis.J Rheumatol. 2007 May;34(5):1133-8. Epub 2007 Mar 1. J Rheumatol. 2007. PMID: 17343315
-
Differentiating macrophage activation syndrome in systemic juvenile idiopathic arthritis from other forms of hemophagocytic lymphohistiocytosis.J Pediatr. 2013 Jun;162(6):1245-51. doi: 10.1016/j.jpeds.2012.11.081. Epub 2013 Jan 17. J Pediatr. 2013. PMID: 23333131
-
The clinical spectrum and treatment options of macrophage activation syndrome in the pediatric age.Eur Rev Med Pharmacol Sci. 2006 Mar-Apr;10(2):53-9. Eur Rev Med Pharmacol Sci. 2006. PMID: 16705949 Review.
Cited by
-
Cytokine Storm Syndrome Associated with Systemic Juvenile Idiopathic Arthritis.Adv Exp Med Biol. 2024;1448:323-353. doi: 10.1007/978-3-031-59815-9_23. Adv Exp Med Biol. 2024. PMID: 39117825 Review.
-
Antiedematogenic and Anti-Inflammatory Activity of the Monoterpene Isopulegol and Its β-Cyclodextrin (β-CD) Inclusion Complex in Animal Inflammation Models.Foods. 2020 May 14;9(5):630. doi: 10.3390/foods9050630. Foods. 2020. PMID: 32423148 Free PMC article.
-
Tocilizumab for the Treatment of COVID-19 Among Hospitalized Patients: A Matched Retrospective Cohort Analysis.Open Forum Infect Dis. 2020 Dec 28;8(1):ofaa598. doi: 10.1093/ofid/ofaa598. eCollection 2021 Jan. Open Forum Infect Dis. 2020. PMID: 33537364 Free PMC article.
-
The Immunology of Macrophage Activation Syndrome.Front Immunol. 2019 Feb 1;10:119. doi: 10.3389/fimmu.2019.00119. eCollection 2019. Front Immunol. 2019. PMID: 30774631 Free PMC article. Review.
-
Investigation into the anti-inflammatory and antigranuloma activity of Colchicum luteum Baker in experimental models.Inflammation. 2012 Jun;35(3):881-8. doi: 10.1007/s10753-011-9389-2. Inflammation. 2012. PMID: 21938464
Publication types
MeSH terms
Substances
LinkOut - more resources
Full Text Sources
Other Literature Sources
Medical
Research Materials
