The main syndromes classified under autoimmune enteropathy include immunodysregulation polyendocrinopathy enteropathy X-linked (IPEX) syndrome due to mutations in FOXP3, IPEX-like syndromes that have the features of IPEX but do not have the FOXP3 mutation (includes mutations in BACH2, CD25, STAT1, STAT3, CTLA-4, ITCH, MALT1, PTPN2 and LRBA) and autoimmune polyglandular syndrome type 1 (APS-1) due to mutations in the AIRE gene. Here, LRBA is linked to immune dysregulation-polyendocrinopathy-enteropathy-X-linked syndrome.