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Autoimmune enteropathy in children and adults
Massimo Montalto1, Ferruccio D'Onofrio, Luca Santoro
1Institute of Internal Medicine, Catholic University, Rome, Italy. mmontalto@rm.unicatt.it
Scandinavian Journal of Gastroenterology
|March 4, 2009
Summary
Autoimmune enteropathy causes severe diarrhea and malabsorption, primarily in children. Management involves nutritional support, immunosuppression, and emerging biological agents for better outcomes.
Area of Science:
- Gastroenterology
- Immunology
- Pediatrics
Background:
- Autoimmune enteropathy (AIE) is a rare condition causing severe diarrhea, malabsorption, and intestinal damage.
- It primarily affects infants and children, but adult cases exist.
- Pathogenesis involves immune dysregulation, with autoantibodies potentially being secondary.
Purpose of the Study:
- To review the characteristics, pathogenesis, prognosis, and management of autoimmune enteropathy.
- To highlight associated autoimmune disorders like IPEX and APECED syndromes.
- To discuss recent therapeutic advancements, including biological agents.
Main Methods:
- Literature review of autoimmune enteropathy cases.
- Analysis of pathogenetic mechanisms and clinical presentations.
- Evaluation of prognostic factors and treatment strategies.
Main Results:
- AIE presents with severe gastrointestinal symptoms and malabsorption.
- Associated autoimmune conditions can influence presentation and severity.
- Prognosis is linked to symptom severity, lesion extent, and extra-intestinal involvement.
Conclusions:
- Autoimmune enteropathy requires comprehensive management including nutritional support and immunosuppression.
- Emerging biological therapies show promise for treating this condition.
- Understanding associated autoimmune disorders is crucial for patient care.
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