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Autoimmune enteropathy.
Last reviewed: 07.07.2025
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Autoimmune enteropathy is a persistent protein-losing diarrhea accompanied by the production of autoantibodies, a sign of active autoimmune T-cell inflammation. Morphologically, it is accompanied by villous atrophy and massive mononuclear infiltration of the lamina propria of the small intestinal mucosa.
There are 3 types of autoimmune enteropathy:
- Type I - /PEX syndrome (immune dysregulation, polyendocrinopathy, enteropathy, X-linked inheritance) - the most severe variant, only boys get sick;
- Type II - /PEX-like syndrome - a milder course, boys and girls get sick;
- Type III - isolated gastrointestinal tract damage.
ICD-10 code
D89.8 Other specified disorders involving the immune mechanism, not elsewhere classified.
Symptoms
Autoimmune enteropathy manifests itself already in the 1-3rd week of life, accompanied by severe diarrhea. Diarrhea is not the only symptom, unlike diseases associated with enterocyte pathology. For /PEX syndrome, the development of diabetes mellitus, glomerulonephritis, hemolytic anemia with a positive Coombs reaction is typical. Stool with an admixture of blood, excretion of plasma proteins and their excretion in feces, a decrease in the content of serum albumin, an increase in the clearance of a1-antitrypsin are characteristic. Signs of a systemic inflammatory reaction are detected, according to the results of a biochemical blood test and coprological examination data, an inflammatory syndrome is present. During an immunohistochemical study, antibodies to enterocyte antigens are found on the surface of enterocytes. In most cases, children die in the first year of life.
Treatment
Complete cessation of enteral nutrition sometimes leads to improvement, but in severe cases diarrhea persists. Systemic glucocorticosteroids and cytostatic drugs are also included in the complex treatment. In some cases, a positive effect is noted after bone marrow transplantation.
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