Germline gain-of-function mutations in CARD11 underlie B-cell expansion with NF-κB and T-cell anergy (BENTA) disease, which is a PID with recurrent respiratory tract and viral infections such as molluscum contagiosum and EBV viremia.227 BENTA patients also display autoimmune lymphoproliferative syndrome (ALPS)-like lymphoproliferation and SLE-like autoimmunity, including autoimmune cytopenias. Here, NFKB1 is linked to BENTA disease.