ArticleClinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association2007
Adult autoimmune enteropathy: Mayo Clinic Rochester experience.
Article in Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 83 papers.
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83 citing papers in PubMed, 286 citations in OpenAlex.
- Mesenteric Panniculitis in a Patient with Ulcerative Colitis in Remission on Vedolizumab Therapy: A Case Report and Literature Review.Journal of clinical medicine · 2026Review
- Diagnostic clues in patients with clinical malabsorption and pathological small intestinal villous atrophy: Immune-mediated type and beyond.World journal of gastroenterology · 2026Review
- Article
- Risk of malignancy and surveillance of premalignant gastrointestinal lesions in adult celiac disease.Frontiers in oncology · 2026Review
- Relieving the discrimination dilemma of adult autoimmune enteropathy and common variable immunodeficiency disease: two rare causes of chronic diarrhea and small intestinal villous atrophy.Clinical rheumatology · 2025Article
- Liver Involvement in Celiac Disease and Immune-Mediated Diseases of the Small Bowel.Liver international : official journal of the International Association for the Study of the Liver · 2025Review
- Diagnostic Challenges in Enteropathies: A Histopathological Review.Diagnostics (Basel, Switzerland) · 2025Review
- Intestinal failure from thymoma-associated autoimmune enteropathy: a rare case report.Clinical journal of gastroenterology · 2025Article
- Treatment of Autoimmune Enteropathy With Vedolizumab.ACG case reports journal · 2025Article
- Comprehensive insights into pathogenesis, diagnosis, treatment, and prognosis in adult autoimmune enteropathy.Orphanet journal of rare diseases · 2025Review
- Non-celiac Enteropathies.Current gastroenterology reports · 2025Review
- Autoimmune enteropathy in adults: a case report and literature review.AME case reports · 2025Article
- Article
- Adult-Onset Autoimmune Enteropathy Mimicking Disaccharidase Deficiency.Gastroenterology research · 2024Article
- Clinical manifestations, diagnosis and long-term prognosis of adult autoimmune enteropathy: Experience from Peking Union Medical College Hospital.World journal of gastroenterology · 2024Article
- Intractable diarrhea in an infant-autoimmune enteropathy: A case report.JPGN reports · 2024Article
- Localised intestinal Kaposi sarcoma in a patient with non-coeliac seronegative villous atrophy.BMJ case reports · 2023Article
- Article
- Adult-Onset Autoimmune Enteropathy: A Case Report.Cureus · 2023Article
- Genetic Diagnosis Guides Treatment of Autoimmune Enteropathy.Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association · 2023Article
23 more citing papers are in PubMed but not listed here.
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Authors and funding
7 authors at 3 institutions in 1 country.
Funding
Abstract
BACKGROUND &
aimsAutoimmune enteropathy is a rare cause of intractable diarrhea associated with circulating gut autoantibodies and a predisposition to autoimmunity. It is rarely observed in adults, with only 11 cases reported to date.
methodsFifteen adults with autoimmune enteropathy were identified at the Mayo Clinic, Rochester, from May 2001-June 2006. The demographic, clinical, and treatment data were abstracted from their records.
resultsThe study population was 87% white, 47% female, with median age of 55 years (interquartile range, 42-67 years). All patients had protracted diarrhea, weight loss, and malnutrition. Celiac disease was excluded by lack of response to gluten-free diet or absence of the celiac disease susceptibility HLA genotypes. Fourteen patients were tested for gut epithelial cell antibodies, and 93% were positive for anti-enterocyte and/or anti-goblet cell antibodies. Predisposition to autoimmune diseases was noted in 80%, as indicated by a variety of circulating autoantibodies. Small intestinal histopathologic findings included subtotal villous atrophy and lymphoplasmacytic infiltration in the lamina propria with relatively few surface intraepithelial lymphocytes. T-cell receptor gene rearrangement studies were negative in all cases. Immunosuppressive therapy was required in 93% of cases. Clinical improvement was noted in 60% after 1-8 weeks of steroid therapy.
conclusionsAutoimmune enteropathy is a heterogeneous disease and should be considered in the differential diagnosis of malabsorption and small bowel villous atrophy. The presence of gut epithelial cell antibodies can help confirm the diagnosis. No single agent is unequivocally effective in inducing remission, and immunosuppressive therapy is required in most cases.
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