Dysregulated stimulation-induced cytokine secretion and especially increased production of TNF can result in autoimmunity.224 Increased production of TNF has been described in patients with heterozygous NFKB1 mutations and may be an additional or alternative explanation for autoimmune or autoinflammatory disease in the context of immunodeficiency due to heterozygous NFKB1 mutations. Here, NFKB1 is linked to immunodeficiency disease.