IKBKG and autoinflammatory syndrome: NEMO-NDAS was recently described as an autoinflammatory syndrome caused by an increased proportion of NEMO-∆ex5 protein due to alternative splicing of IKBKG. The patients reported so far carried IKBKG splice-site variants and presented with autoinflammation including panniculitis, uveitis, hepatitis, lymphoproliferation, and cytopenia [21–23, 26].