In addition to the CD-like phenotype, patients with LO-mIBD caused by ICOS deficiency could be diagnosed with UC, IBD, and CNE; those with TNFAIP3 deficiency could be diagnosed with IBD and intestinal Behçet’s disease (iBD); and those with SYK mutations were characterized by CNE. Here, TNFAIP3 is linked to inflammatory bowel disease.