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Objectives Given the similarities between primary and secondary HLH (sec-HLH), including MAS, we measured levels of IFNg, IFNg-related chemokines (CXCL9, CXCL10, CXLC11), and IL-6 in patients with sec-HLH, and in patients with systemic Juvenile Idiopathic Arthritis (sJIA) with or without MAS at sampling and evaluated their relation to disease activity. In addition, we evaluated the correlation between serum levels of IFNg and of the three IFNg related chemokines with themselves and with laboratory parameters of disease activity in patients with active MAS.

High levels of interferon-gamma (IFNγ) in macrophage activation syndrome (MAS) and CXCL9 levels as a biomarker for IFNγ production in MAS