Traditionally, inflammatory bowel diseases (IBD) ulcerative colitis (UC) and Crohn’s disease (CD). At the same time, there are a number of lesions of the gastrointestinal tract, which can proceed for a long time under the guise of IBD, masking the true cause of the disease. This leads to late diagnosis and, quite often, fatal consequences. These diseases include autoimmune enteropathy (AIE). It is a fairly rare disease characterized by severe diarrhea and immune- mediated damage to the intestinal mucosa.The aim is to describe the criteria of diagnosis, etiology, pathogenesis, epidemiology, clinic and treatment of AIE in children based on an analysis of modern literature.Results. Diagnostic criteria for AIE include chronic diarrhea (lasting more than 6 weeks), malabsorption syndrome, specific histological findings from small bowel biopsy with the exclusion of other causes of villous atrophy. An additional criterion is the presence of antibodies against enterocytes or goblet cells. There are: (1) AIEs associated with syndromes such as IPEX and APECED; (2) an isolated form of GI AIE with antibodies against enterocytes without diseases of the digestive system; and (3) any form of AIE in girls associated with any other autoimmune phenomena. To date, at least five subtypes of AIE are known: Primary AIE (pediatric); Syndromic AIE (pediatric); Primary (sporadic) EIA of adults; Secondary (iatrogenic) AIE of adults; Paraneoplastic AIE. Patients with AIE may have associated autoimmune diseases, including diabetes mellitus, autoimmune hepatitis, alopecia, hypothyroidism, and interstitial nephritis. AIE. is a complex disease and potentially life-threatening, the mortality rate reaches 30% in pediatric practice. The prognosis depends on the age of onset of the disease, the severity of symptoms and the degree of histological damage to the gastrointestinal tract. Along with nutritional support, immunosuppressive therapy, the use of therapy based on modern knowledge in the field of molecular biology can help control the disease.

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