+ Site Statistics
+ Search Articles
+ PDF Full Text Service
How our service works
Request PDF Full Text
+ Follow Us
Follow on Facebook
Follow on Twitter
Follow on LinkedIn
+ Subscribe to Site Feeds
Most Shared
PDF Full Text
+ Translate
+ Recently Requested

Macrophage Activation Syndrome: different mechanisms leading to a one clinical syndrome



Macrophage Activation Syndrome: different mechanisms leading to a one clinical syndrome



Pediatric Rheumatology Online Journal 15(1): 5



Macrophage activation syndrome (MAS) is a severe complication of rheumatic disease in childhood, particularly in systemic Juvenile Idiopathic Arthritis (sJIA). It is characterize by an uncontrolled activation and proliferation of T lymphocytes and macrophages. MAS is currently classified among the secondary or acquired forms of haemophagocytic lymphohistiocytosis (sHLH). The reason is that MAS shares clinical and laboratory features with primary genetic HLH (pHLH). In this context is conceivable that some of the pathogenic mechanisms of pHLH may be involved in other forms of HLH. Heterozygosity for mutations of genes involved in pHLH may lead to a cytotoxic defect and to a development of clinical overt disease. But other different contributors might be involved to the development of MAS such as infections or underlying inflammation. In MAS, the inflammatory status of the patient is a major contributor of the disease. Indeed, the majority of the MAS episodes occurs during active disease phases or at disease onset. In addition, recent evidence in animals and humans suggest that genetics may also play a major role in contributing to hyperinflammation and particularly to macrophages hyper-responses. We hypothesize that HLH may be one unique clinical syndrome, to whose generation different mechanisms may contribute, and maintained by one final effector mechanism.

Please choose payment method:






(PDF emailed within 0-6 h: $19.90)

Accession: 059935564

Download citation: RISBibTeXText

PMID: 28095869

DOI: 10.1186/s12969-016-0130-4


Related references

Autoimmune/inflammatory syndrome leading to macrophage activation syndrome: An example of autoinflammatory spectrum disorder?. Israel Medical Association Journal 18(9): 571, 2017

Visceral leishmaniasis leading to macrophage activation syndrome. Medecine et Maladies Infectieuses 2019, 2019

Macrophage activation syndrome leading to fatality in subcutaneous panniculitis-like T cell lymphoma. Annals of Hematology 93(5): 873-875, 2014

Macrophage Activation-Like Syndrome: A Distinct Entity Leading to Early Death in Sepsis. Frontiers in Immunology 10: 55, 2019

The hyperferritinemic syndrome: macrophage activation syndrome, Still's disease, septic shock and catastrophic antiphospholipid syndrome. Bmc Medicine 11: 185, 2014

A hyper-ferritinemia syndrome evolving in recurrent macrophage activation syndrome, as an onset of amyopathic juvenile dermatomyositis: a challenging clinical case in light of the current diagnostic criteria. Autoimmunity Reviews 13(11): 1142-1148, 2015

The link between Kawasaki disease shock syndrome and macrophage activation syndrome in terms of organ dysfunction observed in children with systemic inflammatory response syndrome. Paediatrics and International Child Health 38(4): 308-309, 2018

Secondary hemophagocytic lymphohistiocytosis and severe sepsis/ systemic inflammatory response syndrome/multiorgan dysfunction syndrome/macrophage activation syndrome share common intermediate phenotypes on a spectrum of inflammation. Pediatric Critical Care Medicine 10(3): 387-392, 2009

Development and Initial Validation of the Macrophage Activation Syndrome/Primary Hemophagocytic Lymphohistiocytosis Score, a Diagnostic Tool that Differentiates Primary Hemophagocytic Lymphohistiocytosis from Macrophage Activation Syndrome. Journal of Pediatrics 189: 72-78.E3, 2017

Plasma exchange, methylprednisolone, IV immune globulin, and now anakinra support continued PICU equipoise in management of hyperferritinemia-associated sepsis/multiple organ dysfunction syndrome/macrophage activation syndrome/secondary hemophagocytic lymphohistiocytosis syndrome*. Pediatric Critical Care Medicine 15(5): 486-488, 2015

Clinical and laboratory features of macrophage activation syndrome. Zhongguo Dang Dai Er Ke Za Zhi 19(2): 188-192, 2017

Considerations in clinical diagnosis of childhood macrophage activation syndrome. Zhonghua Er Ke Za Zhi 44(11): 839-840, 2007

Clinical features and correct diagnosis of macrophage activation syndrome. Expert Review of Clinical Immunology 11(9): 1043-1053, 2016

An update on renal involvement in hemophagocytic syndrome (macrophage activation syndrome). Journal of Nephropathology 5(1): 8-14, 2016

First report of macrophage activation syndrome in hyperimmunoglobulinemia D with periodic fever syndrome. Arthritis and Rheumatism 56(2): 658-661, 2007