Inflammatory bowel disease has been identified as a possible sign of several recently discovered PIDs; it may be a clinical feature of a multisystemic disease or a consequence of epithelial barrier defects, phagocytic defects, B and T cell abnormalities (severe combined immunodeficiency, common variable immunodeficiency, Wiskott–Aldrich syndrome), autoimmunity (XLP2), and IL10R defects (19–21). Here, IL10RA is linked to severe combined immunodeficiency.