We found that TNF-dependent NF-kB signaling reduces the threshold for IFN-γ-mediated inflammatory responses in Blau syndrome and that resetting of this primed state by anti-TNF treatment contributes to the prevention of the autoinflammatory loop, even in the presence of a NOD2 mutation and IFN-γ stimulation. This evidence concerns the gene NFKB1 and Blau syndrome.