Yet, elevated BAFF levels have been shown to inhibit negative selection of autoreactive B cells, in CVID autoimmunity as in other diseases, which apparently contributes to the increased autoimmunity seen in CVID patients with mutations in the BAFF receptor transmembrane activator and calcium-modulator and cyclophilin ligand interactor (TACI) (70–72). Here, TNFSF13B is linked to common variable immunodeficiency.