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Deprecated: Implicit conversion from float 209.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534 Best+Pract+Res+Clin+Rheumatol 2014 ; 28 (2): 277-92 Nephropedia Template TP
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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 PMID24974063show ga
Macrophage activation syndrome (MAS) is an episode of overwhelming inflammation that occurs most commonly in children with systemic juvenile idiopathic arthritis. 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 proinflammatory 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.