Expert Insights on MAS: sJIA/AOSD and Macrophage Activation Syndrome

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  • เผยแพร่เมื่อ 21 พ.ย. 2024

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  • @simonsteen-andersen3181
    @simonsteen-andersen3181 4 หลายเดือนก่อน

    Is the increased IFN-gamma production ISG15-induced (ISG15/CD11a/IFN-g) and thus independent of Il-12 receptor chain upregulation,- or IL-12-induced with upregulation of both IL12RB1 and IL12RB2?
    This question is interesting because there have been cases of macrophage activation syndrome/lymphohistiocytosis also in patients with IL12RB1 deficiency (MSMD) who also display decreased perforin expression and decreased clearance of viral pathogens as well as decreased FAS/FASL-dependent apoptosis, triggered from viral infection. And, of course, these patients exhibit an abolished IL-12/IFN-gamma circuit.