Mutations in the lipopolysaccharide responsive beige-like anchor protein (LRBA) gene cause a common variable immunodeficiency (CVID)-like disease with predominant antibody deficiency (hypogammaglobulinemia) and autoimmunity (e.g., autoimmune hemolytic anemia as well as atrophic gastritis with autoantibodies against intrinsic factor, autoimmune enteropathy, hypothyroidism, myasthenia gravis, polyarthritis), and inflammatory bowel disease (80, 194, 195). Here, LRBA is linked to autoimmune enteropathy.