Genetic alterations in Lipopolysaccharide-responsive BEACH and anchor containing protein (LRBA) are associated with early-onset common variable immunodeficiency (CVID), hypogammaglobulinemia and autoimmunity manifested in variable clinical phenotypes such as respiratory infections and inflammatory bowel disease (IBD) [9,10,11].We have shown previously that DM was seen in LRBA-deficient patients with and without detected β-related autoantibodies [12]. Here, LRBA is linked to common variable immunodeficiency.