This approach resulted in the identification of two previously described gene variants in NFKB1 (21, 32), nine novel variants, including variants in STAT3, TNFAIP3, IL-17F, IRAK4, TTC37, DDX41, NLRC3, TNFRSF1A, and PLCG2 (22–31, 34–36) associated with different states of immune dysregulation but not previously associated with CVID, and with possible probable disease-causing impact according to bioinformatics. Here, SKIC3 is linked to common variable immunodeficiency.