Abstract
Autoimmune enteropathy (AIE) is a rare disease characterised by severe diarrhoea and immune-mediated damage of the intestinal mucosa. The objective: based on analysis of modern literature to describe the diagnostic criteria, etiology, pathogenesis, epidemiology, clinical features and treatment of AIE in children. Results. The diagnostic criteria of AIE include chronic diarrhoea (lasting more than 6 weeks), malabsorption syndrome, specific histological findings of small intestine biopsy when other causes of villous atrophy are excluded. An additional criterion is the presence of antibodies against enterocytes or goblet cells. The following types are singled out: (1) AIE associated with such syndromes as IPEX and APECED; (2) an isolated form of GI AIE with the presence of anti-enterocyte antibodies without diseases of the digestive system; and (3) any form of AIE in girls associated with any other autoimmune phenomena. At present, not less than five subtypes of AIE are known: Primary AIE (paediatric); Syndromal AIE (paediatric); Primary (sporadic) adult ОВОС AIE; Secondary (iatrogenic) adult AIE; Paraneoplastic AIE. Patients with AIE might have associated autoimmune diseases, including diabetes mellitus, autoimmune hepatitis, alopecia, hypothyroidism, and interstitial nephritis. AIE is a complex and potentially life-threatening disease, with the mortality rates reaching 30% in paediatric practice. The prognosis depends on the age of the disease onset, the severity of symptoms and histological lesions of the gastrointestinal tract. The use of therapy based on modern molecular biology technologies, along with nutritive support, immunosuppressive therapy, can help control the disease. Key words: autoimmune enteropathy, IPEX syndrome
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