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Autoimmune enteropathy
Last reviewed: 23.04.2024
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Autoimmune enteropathy is a persistent protein-losing diarrhea accompanied by autoantibody production, a sign of active autoimmune T-cell inflammation. Morphologically accompanied by atrophy of villi and massive mononuclear infiltration of the lamina propria of the small intestine mucosa.
There are 3 types of autoimmune enteropathy:
- I type - / REX-syndrome (immune dysregulation, polyendocrinopathy, enteropathy, X-linked type of inheritance) - the most difficult variant, only boys are ill;
- Type II - / REX-like syndrome - a softer course, sick boys and girls;
- III type - isolated gastrointestinal lesion.
ICD-10 code
D89.8. Other specified disorders involving the immune mechanism, not elsewhere classified.
Symptoms
Autoimmune enteropathy manifests itself on the 1-3 th week of life, accompanied by severe diarrhea. Diarrhea is not the only symptom, unlike diseases associated with pathology of enterocytes. For / REX syndrome, the development of diabetes mellitus, glomerulonephritis, hemolytic anemia with a positive Coombs reaction is typical. A stool with an admixture of blood is characteristic, the excretion of plasma proteins and excretion of them in the feces, a decrease in serum albumin content, an increase in the clearance of a1-antitrypsin. There are signs of a systemic inflammatory reaction, according to the results of a biochemical blood test and the data of a coprologic study, there is an inflammatory syndrome. When immunohistochemical studies on the surface of enterocytes, antibodies to antigens of enterocytes are detected. In most cases, children die in the first year of life.
Treatment
Complete cancellation of enteral nutrition sometimes leads to improvement, but with severe current, diarrhea persists. Also in the complex treatment include systemic glucocorticosteroids and cytotoxic drugs. In some cases, a positive effect is noted after the operation of bone marrow transplantation.
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