Abstract
Autoimmune enteropathy (AIE) is a rare immune mediated disorder primarily affecting children, characterized by chronic diarrhea, malabsorption, vomiting, weight loss and villous atrophy. It has also been observed in adults presenting diagnostic and treatment challenges due to its overlap with other gastrointestinal disorders such as celiac disease. Initial diagnostic criteria for AIE include small bowel villous atrophy, lack of response to dietary restrictions, presence of anti-enterocyte antibodies, and predisposition to autoimmunity without severe immunodeficiency. Refined criteria emphasize characteristic histological findings and exclusion of other causes of villous atrophy. AIE is associated with various autoimmune disorders and can present with overlapping features with Celiac disease, including villous atrophy but without significant intraepithelial lymphocytosis. Treatment primarily involves immunosuppression using corticosteroids, calcineurin inhibitors, and anti-tumor necrosis factor therapy, alongside nutritional support. Despite the challenges, understanding AIE's diverse manifestations and improving diagnostic criteria are essential for effective management and improved patient outcome. Further research is needed to elucidate the pathogenesis, disease progression and long-term outcomes of AIE.