Mutations in NFKB2 have been previously associated with common variable immunodeficiency (CVID) (Chen et al. 2013; Lindsley et al. 2014; Liu et al. 2014), a heterogeneous disorder in which 25% of patients suffer autoimmune disorders, including thrombocytopenic purpura, autoimmune hemolytic anemia, rheumatoid arthritis, and autoimmune enteropathy (which can be classified as Crohn's disease) (Cunningham-Rundles 2008; Lopez-Herrera et al. 2012). Here, NFKB2 is linked to autoimmune enteropathy.