Interleukin-18 diagnostically distinguishes and pathogenically promotes human and murine macrophage activation syndrome

著者: Eric S. Weiss, Charlotte Girard, Dirk Holzinger, Adriana A. de Jesus, Zeshan Tariq, Jennifer Picarsic, Eduardo J. Schiffrin, Dirk Foell, Alexei A. Grom, Sandra Ammann, Stephan Ehl, Tomoaki Hoshino, Raphaela Goldbach‐Mansky, Cem Gabay, Scott Canna - Blood 2018 被引用: 395

mice, whose free IL-18 levels were normal, did not. Thus, we describe a unique connection between MAS risk and chronic IL-18, identify epithelial inflammasome hyperactivity as a potential source, and demonstrate the pathogenicity of free IL-18. These data suggest an IL-18-driven pathway, complementary to the cytotoxic impairment of fHLH, with potential as a distinguishing biomarker and therapeutic target in MAS.