PID due to damaging monoallelic variants in NFKB2 leads to immunodeficiency characterized by recurrent respiratory tract infections and failed control of herpesviruses.32 In addition to immunodeficiency, the vast majority (~80%) of patients display at least one autoimmune manifestation, including autoimmune cytopenias, arthritis, and alopecia. Here, NFKB2 is linked to Immunodeficiency.