Overall, we conclude that CVID subjects with currently identifiable gene variants, either associated with or causative of this immune defect, appear to have an increased numbers of autoimmune manifestations, more significant respiratory disease and granulomatous changes in pathology; some of these differences can be attributed to co-existence of TACI variants as a genetic modifier. The gene discussed is TNFRSF13B; the disease is common variable immunodeficiency.