Christodoulidis G, Agko SE, Kouliou MN, Koumarelas KE, Zacharoulis D. Advances and challenges in diagnosing and managing adult autoimmune enteropathy. World J Gastroenterol 2025; 31(2): 99118 [DOI: 10.3748/wjg.v31.i2.99118]
Corresponding Author of This Article
Grigorios Christodoulidis, PhD, Executive Associate Editor-in-Chief, Department of General Surgery, University Hospital of Larissa, Mezourlo, Larissa 41110, Greece. gregsurg@yahoo.gr
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Letter to the Editor
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Gastroenterol. Jan 14, 2025; 31(2): 99118 Published online Jan 14, 2025. doi: 10.3748/wjg.v31.i2.99118
Advances and challenges in diagnosing and managing adult autoimmune enteropathy
Grigorios Christodoulidis, Sara E Agko, Marina N Kouliou, Konstantinos E Koumarelas, Dimitris Zacharoulis
Grigorios Christodoulidis, Marina N Kouliou, Department of General Surgery, University Hospital of Larissa, Larissa 41110, Greece
Sara E Agko, Department of Intensive Care Unit, Asklepios Paulinen Clinic Wiesbaden, Wiesbaden 65197, Germany
Konstantinos E Koumarelas, Department of Emergency Medicine, General Hospital of Larissa, Larissa 41221, Greece
Dimitris Zacharoulis, Department of Surgery, University Hospital of Larissa, Larissa 41334, Greece
Author contributions: Christodoulidis G, Agko SE, Koumarelas KE, Kouliou MN and Zacharoulis D contributed to this paper; Christodoulidis G designed the overall concept and outline of the manuscript; Christodoulidis G, Agko SE, Koumarelas KE, Kouliou MN and Zacharoulis D contributed to the discussion and design of the manuscript, writing and editing the manuscript, and review of the literature.
Conflict-of-interest statement: The authors declare that they have no conflicts of interest.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Grigorios Christodoulidis, PhD, Executive Associate Editor-in-Chief, Department of General Surgery, University Hospital of Larissa, Mezourlo, Larissa 41110, Greece. gregsurg@yahoo.gr
Received: July 14, 2024 Revised: November 1, 2024 Accepted: November 14, 2024 Published online: January 14, 2025 Processing time: 156 Days and 23.4 Hours
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.
Core Tip: Autoimmune enteropathy (AIE) is a rare, immune-mediated disorder predominantly affecting children, characterized by chronic diarrhea, malabsorption, vomiting, weight loss, and villous atrophy. Adults can also be affected, complicating diagnosis and treatment due to symptom overlap with other gastrointestinal disorders. Key diagnostic criteria include small bowel villous atrophy, lack of response to dietary restrictions, presence of anti-enterocyte antibodies, and a predisposition to autoimmunity without severe immunodeficiency. Further research is needed to elucidate the pathogenesis, disease progression and long-term outcomes of AIE.
