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2014 ; 28
(2
): 277-92
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Macrophage activation syndrome and cytokine-directed therapies
#MMPMID24974063
Schulert GS
; Grom AA
Best Pract Res Clin Rheumatol
2014[Apr]; 28
(2
): 277-92
PMID24974063
show ga
Macrophage activation syndrome (MAS) is an episode of overwhelming inflammation
that occurs most commonly in children with systemic juvenile idiopathic arthritis
(SJIA). It is characterized by expansion and activation of T lymphocytes and
hemophagocytic macrophages and bears great similarity to hemophagocytic
lymphohistiocytosis (HLH). This disorder has substantial morbidity and mortality,
and there is frequently a delay in recognition and initiation of treatment. Here,
we will review what is known about the pathogenesis of MAS and, in particular,
its similarities to HLH. The development of MAS is characterized by a cytokine
storm, with the elaboration of numerous pro-inflammatory cytokines. We will
examine the evidence for various cytokines in the initiation and pathogenesis of
MAS and discuss how new biologic therapies may alter the risk of MAS. Finally, we
will review current treatment options for MAS and examine how cytokine-directed
therapy could serve as novel treatment modalities.