{
  "abstract": "Introduction: Enteropathy is a significant and under-recognised complication of inborn errors of immunity (IEI), frequently misclassified as inflammatory bowel disease (IBD) or coeliac disease due to overlapping and non-specific histopathological findings. Diagnostic criteria remain poorly defined, contributing to substantial diagnostic delay and associated morbidity.\n\nMethods: We conducted a structured literature review and established the AT-SEA (Adult Tertiary Severe Enteropathy Audit) working group, a collaborative network of Australian clinical immunologists operating under the Clinical Immunogenomics Research Consortium Australasia (CIRCA), to characterise the clinicopathological features of IEI-associated enteropathy across a multicentre cohort.\n\nResults: Twenty-eight patients were identified. Moderate or severe disease was present in 89%, with malabsorption in 71% and protein-losing enteropathy in 43%. Across 48 biopsy reports, the most frequent histopathological patterns were coeliac-like changes (including villous atrophy, increased intraepithelial lymphocytes and crypt hyperplasia), IBD-like changes, reduced or absent plasma cells, graft versus host disease (GVHD)-like changes or increased apoptotic bodies and intraepithelial neutrophilia. Seventy-seven percent of patients demonstrated two or more concurrent patterns. Increasing histological complexity correlated significantly with clinician-assessed disease severity (Kruskal-Wallis p=0.042).\n\nDiscussion: IEI-associated enteropathy is characterised by overlapping and evolving histopathological patterns rather than discrete subtypes. The co-occurrence of multiple patterns should prompt consideration of an underlying IEI.",
  "abstract_shown": true,
  "affiliations": [
    "Faculty of medicine and health, University of Sydney",
    "Immunology Department, Westmead Hospital",
    "Centre for Immunology and Allergy Research, Westmead Institute of Medical Research",
    "Clinical Immunogenomics Research Consortium Australasia (CIRCA)",
    "Immunology and Allergy Unit, Monash Health",
    "Monash University",
    "SydPath",
    "The Garvan Institute of Medical Research",
    "University of New South Wales",
    "Immunology Department, St Vincent’s Hospital",
    "Immunology Department, Blacktown Hospital",
    "Western Sydney University",
    "Institute of Clinical Pathology and Medical Research",
    "Immunology Department, Queensland Children’s Hospital",
    "University of Queensland",
    "Griffith University",
    "Immunology Department, Royal Hobart Hospital",
    "Murdoch Children’s Research Institute",
    "Immunology Department, Campbelltown Hospital",
    "Immunology Department, Alfred Health",
    "The Jeffrey Modell Diagnostic and Research Centre for Primary Immunodeficiencies"
  ],
  "article_type": "research article",
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      "name": "Meera Thangarajah"
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      "name": "Samar Ojaimi"
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    {
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        7,
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      "name": "Alisa Kane"
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      "name": "Sanjay Swaminathan"
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      "name": "Ming Wei Lin"
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      "name": "Alberto Pinzon-Charry"
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      "name": "Tiarni Templeton"
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    {
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      "name": "Peter McNaughton"
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    {
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      ],
      "name": "Thanh-Thao Adriana Le"
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    {
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      "name": "Constance H. Katelaris"
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      "name": "Julian Bosco"
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    {
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      ],
      "name": "Lucinda J. Berglund"
    }
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  "bibtex": "https://research.draconitic.com/paper/7dyc76d3fwxfs.bib",
  "cited_by": 0,
  "doi": "10.3389/fimmu.2026.1934715",
  "fulltext_url": "https://www.ncbi.nlm.nih.gov/pmc/articles/PMC13716641/?tool=EBI",
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  "landing_url": "https://www.frontiersin.org/articles/10.3389/fimmu.2026.1934715/full",
  "language": "eng",
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  "metadata_licence": null,
  "native_subjects": [
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  "pdf_url": "https://www.frontiersin.org/journals/immunology/articles/10.3389/fimmu.2026.1934715/pdf",
  "pmcid": "PMC13716641",
  "pmid": "42959048",
  "published": "2026-10-15",
  "publisher": "Frontiers Media SA",
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  "subjects": [
    {
      "field": "biology",
      "origin": "openalex:2403|Immunodeficiency and Autoimmune Disorders",
      "subfield": "immunology"
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  "title": "Enteropathy in inborn and acquired errors of immunity: histological patterns and clinical correlates",
  "title_text": "Enteropathy in inborn and acquired errors of immunity: histological patterns and clinical correlates",
  "type": "peer-reviewed",
  "updated_at": "2026-11-02T12:21:07Z",
  "url": "https://research.draconitic.com/paper/7dyc76d3fwxfs",
  "venue": "Frontiers in Immunology",
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}