A117 A CASE OF ADULT AUTOIMMUNE ENTEROPATHY AND PRIMARY SCLEROSING CHOLANGITIS
Bibliographic record
Abstract
Abstract Background Autoimmune enteropathy (AIE) is a rare, immune-mediated condition causing severe diarrhea and mucosal damage. The diagnostic criteria are chronic diarrhea with malabsorption, blunting of the small bowel villi, specific histologic changes, and the exclusion of other causes of villous atrophy. Anti-enterocyte and anti-goblet cell antibodies are supportive of the diagnosis, but not specific or sensitive enough to make the diagnosis. It has several clinical and histologic phenotypes and affects children more than adults. To our knowledge, there are only 2 published cases of AIE with concurrent primary sclerosing cholangitis (PSC). Aims To review the literature for adult AIE, any previous cases of concurrent PSC, and present a case of both conditions. Methods We searched PubMed for “autoimmune enteropathy psc”, “autoimmune enteropathy”, “autoimmune enteropathy case report”, “autoimmune enteropathy adult” and collected all the relevant articles. Relevant clinical features of demographics, IBD status, liver diseases, immune conditions, antibody status, and treatment agents were summarized. Results A 31-year-old man presented with a 20-year history of diarrhea and 11kg weight loss over 3 months. He had gout, no regular medications, and relevant no family history. All initial investigations were normal. Repeat endoscopy showed increased intra-epithelial lymphocytes (IEL) in the colon consistent with lymphocytic colitis. He was given budesonide with moderate improvement. He then developed biliary stasis, and was found to have early PSC. Repeat colonoscopy for ongoing diarrhea showed increased colonic IEL. Ulcerative colitis (UC) was suspected, and he was given oral 5-ASA and prednisone, with subsequent switch to vedolizumab. He then relapsed, requiring IV fluids, total parenteral nutrition (TPN), and IV steroids. Repeat endoscopy showed persistent colonic IEL, and villous blunting in the terminal ileum and duodenum. Celiac disease, IBD, combined variable immunodeficiency (CVID), and enteric infections were excluded. In this context, AIE was considered. He improved with IV steroids, and was switched to infliximab. Despite initial improvement, he had worsening diarrhea and malnutrition. After dose escalation of infliximab, and further IV steroids and TPN, he was started on a trial of ustekinumab. We found 35 articles that reported on 100 adult cases of AIE. There were two previous adult cases of concurrent PSC. We found 7 cases where TNF-alpha inhibitors were used, one case where vedolizumab was used, and no cases using ustekinumab. Details are in Table 1. Conclusions AIE is a rare condition causing chronic diarrhea and gut mucosal damage reported only in case reports and series. There have only been 2 other reported cases of concurrent PSC with AIE in adults. This is the first reported case of ustekinumab being tried to control symptoms. Funding Agencies None
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How this classification was reachedexpand
Full frame machine prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. The Gemma side is a direct model label for every work in the frame, read from the title-only record. The Codex side is a classifier learned from the 10,348 direct Codex labels and calibrated to design-weighted sample rates; fields without enough sample support carry no Codex call. Candidate is the union of the two sides; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels.
Distilled classifier scores by category (both heads)
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.001 | 0.004 |
| Meta-epidemiology (narrow) | 0.001 | 0.001 |
| Meta-epidemiology (broad) | 0.001 | 0.002 |
| Bibliometrics | 0.009 | 0.006 |
| Science and technology studies | 0.001 | 0.001 |
| Scholarly communication | 0.002 | 0.003 |
| Open science | 0.001 | 0.001 |
| Research integrity | 0.003 | 0.001 |
| Insufficient payload (model declined to judge) | 0.007 | 0.002 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from itClassification
machine, unvalidatedMachine predicted; a candidate call from one source (direct Gemma or distilled Codex), not a consensus.
How this classification was reached, model by model and score by score, is at the end of the page under "How this classification was reached".