Autoimmune enteropathy
Autoimmune enteropathy is a rare autoimmune disorder characterized by weight loss from malabsorption, severe and protracted diarrhea, and autoimmune damage to the intestinal mucosa. Autoimmune enteropathy typically occurs in infants and younger children however, adult cases have been reported in literature. Autoimmune enteropathy was first described by Walker-Smith et al. in 1982.
| Autoimmune enteropathy | |
|---|---|
| Other names | Severe immune-mediated enteropathy, Immune-mediated protracted diarrhea of infancy |
| Histological evidence of enteropathy (inflammatory infiltrate, villus blunting) seen in this intestinal biopsy from a child with malnutrition. | |
| Specialty | Immunology Gastroenterology |
| Symptoms | Diarrhea, and autoimmune damage to the intestinal mucosa. |
| Complications | Electrolyte imbalances, malabsorption, and failure to thrive. |
| Usual onset | First six months of life. |
| Duration | Chronic. |
| Diagnostic method | histological changes, serologic testing, and clinical signs and symptoms. |
| Differential diagnosis | Graft-versus-host disease, Crohn's disease, celiac disease and lactose intolerance. |
| Treatment | Parenteral nutrition and corticosteroids. |
| Prognosis | 30% mortality rate without treatment. |
| Frequency | <1 in 100,000 infants. |
The mechanisms of autoimmune enteropathy isn't well known but dysfunction or deficiency of CD25+CD4+ regulatory T cells may play a role. Numerous other illnesses and syndromes are linked to autoimmune enteropathy, the most prominent being Autoimmune polyendocrine syndrome type 1 and immune dysregulation polyendocrinopathy enteropathy X-linked (IPEX) syndrome.
Clinical symptoms, laboratory results, and the histological characteristics of a small bowel biopsy are used to make the diagnosis. These patients typically don't respond to diet modification and often require immune-suppressants and sometimes require total parenteral nutrition. The prevalence of autoimmune enteropathy is estimated to be less than 1 in 100,000 infants
The prognosis of autoimmune enteropathy varies and depends on systemic manifestations, the severity of symptoms, and the degree of gastrointestinal involvement. Children suffering from autoimmune enteropathy are frequently vulnerable to systemic and local infections pertaining to immunotherapy, the intestinal and skin barriers, and malnourishment.