Seronegative autoimmune enteropathy with duodenal sparing and colonic clues in an adult female

Chong, Albert C.; Kashani, Amir; Ansstas, Michael; Jamil, Laith H.; Guindi, Maha

doi:10.1007/s12328-020-01336-9

Cited by 3 publications

(1 citation statement)

References 36 publications

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“…A review of the literature revealed that 40 cases showed decreased or absent goblet cells, and 20 cases showed abnormal Paneth cells. In one of these patients, duodenal biopsy lacked other typical findings and only showed only decreased goblet cells[ 13 ]. In fact, abnormalities in the number of goblet and Paneth cells in our cohort are very common, with a higher frequency than currently reported cases in the literature (8%-60%)[ 31 ] and contrary to the findings of another cohort[ 10 ], which might be due to the ignorance of goblet cells or Paneth cells in many studies.…”

Section: Discussionmentioning

confidence: 99%

Clinical manifestations, diagnosis and long-term prognosis of adult autoimmune enteropathy: Experience from Peking Union Medical College Hospital

Li,

Ruan,

Zhou

et al. 2024

World J Gastroenterol

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BACKGROUND Autoimmune enteropathy (AIE) is a rare disease whose diagnosis and long-term prognosis remain challenging, especially for adult AIE patients. AIM To improve overall understanding of this disease’s diagnosis and prognosis. METHODS We retrospectively analyzed the clinical, endoscopic and histopathological characteristics and prognoses of 16 adult AIE patients in our tertiary medical center between 2011 and 2023, whose diagnosis was based on the 2007 diagnostic criteria. RESULTS Diarrhea in AIE patients was characterized by secretory diarrhea. The common endoscopic manifestations were edema, villous blunting and mucosal hyperemia in the duodenum and ileum. Villous blunting (100%), deep crypt lymphocytic infiltration (67%), apoptotic bodies (50%), and mild intraepithelial lymphocytosis (69%) were observed in the duodenal biopsies. Moreover, there were other remarkable abnormalities, including reduced or absent goblet cells (duodenum 94%, ileum 62%), reduced or absent Paneth cells (duodenum 94%, ileum 69%) and neutrophil infiltration (duodenum 100%, ileum 69%). Our patients also fulfilled the 2018 diagnostic criteria but did not match the 2022 diagnostic criteria due to undetectable anti-enterocyte antibodies. All patients received glucocorticoid therapy as the initial medication, of which 14/16 patients achieved a clinical response in 5 (IQR: 3-20) days. Immunosuppressants were administered to 9 patients with indications of steroid dependence (6/9), steroid refractory status (2/9), or intensified maintenance medication (1/9). During the median of 20.5 months of follow-up, 2 patients died from multiple organ failure, and 1 was diagnosed with non-Hodgkin’s lymphoma. The cumulative relapse-free survival rates were 62.5%, 55.6% and 37.0% at 6 months, 12 months and 48 months, respectively. CONCLUSION Certain histopathological findings, including a decrease or disappearance of goblet and Paneth cells in intestinal biopsies, might be potential diagnostic criteria for adult AIE. The long-term prognosis is still unsatisfactory despite corticosteroid and immunosuppressant medications, which highlights the need for early diagnosis and novel medications.

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Section: Discussionmentioning

confidence: 99%

Clinical manifestations, diagnosis and long-term prognosis of adult autoimmune enteropathy: Experience from Peking Union Medical College Hospital

Li,

Ruan,

Zhou

et al. 2024

World J Gastroenterol

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Adult-onset autoimmune enterocolopathy initially presenting with lower gastrointestinal histologic findings: A case report and review of literature

Pfeiffer,

Kennedy

2024

Human Pathology Reports

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Advances and challenges in diagnosing and managing adult autoimmune enteropathy

Christodoulidis,

Agko,

Kouliou

et al. 2025

World J Gastroenterol

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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.

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Seronegative autoimmune enteropathy with duodenal sparing and colonic clues in an adult female

Cited by 3 publications

References 36 publications

Clinical manifestations, diagnosis and long-term prognosis of adult autoimmune enteropathy: Experience from Peking Union Medical College Hospital

Clinical manifestations, diagnosis and long-term prognosis of adult autoimmune enteropathy: Experience from Peking Union Medical College Hospital

Adult-onset autoimmune enterocolopathy initially presenting with lower gastrointestinal histologic findings: A case report and review of literature

Advances and challenges in diagnosing and managing adult autoimmune enteropathy

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