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Peer-reviewedCC BY 4.0Frontiers in Immunology

Enteropathy in inborn and acquired errors of immunity: histological patterns and clinical correlates

  • Meera Thangarajah1,2,3,4
  • Samar Ojaimi4,5,6
  • Alisa Kane4,7,8,9,10
  • Sanjay Swaminathan1,2,3,11,12
  • Ming Wei Lin1,2,3,4,13
  • Alberto Pinzon-Charry4,14,15,16
  • Tiarni Templeton4,14,15
  • Peter McNaughton4,14,15
  • Thanh-Thao Adriana Le4,17
  • Constance H. Katelaris4,12,18,19
  • Julian Bosco4,6,20,21
  • Lucinda J. Berglund1,2,3,4,13
21 affiliations
  1. 1Faculty of medicine and health, University of Sydney
  2. 2Immunology Department, Westmead Hospital
  3. 3Centre for Immunology and Allergy Research, Westmead Institute of Medical Research
  4. 4Clinical Immunogenomics Research Consortium Australasia (CIRCA)
  5. 5Immunology and Allergy Unit, Monash Health
  6. 6Monash University
  7. 7SydPath
  8. 8The Garvan Institute of Medical Research
  9. 9University of New South Wales
  10. 10Immunology Department, St Vincent’s Hospital
  11. 11Immunology Department, Blacktown Hospital
  12. 12Western Sydney University
  13. 13Institute of Clinical Pathology and Medical Research
  14. 14Immunology Department, Queensland Children’s Hospital
  15. 15University of Queensland
  16. 16Griffith University
  17. 17Immunology Department, Royal Hobart Hospital
  18. 18Murdoch Children’s Research Institute
  19. 19Immunology Department, Campbelltown Hospital
  20. 20Immunology Department, Alfred Health
  21. 21The Jeffrey Modell Diagnostic and Research Centre for Primary Immunodeficiencies
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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.

Methods: 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.

Results: 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).

Discussion: 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.

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Cite

BibTeXRIS
BibTeX
@article{thangarajah2026enteropathy,
  title = {{Enteropathy in inborn and acquired errors of immunity: histological patterns and clinical correlates}},
  author = {Meera Thangarajah and Samar Ojaimi and Alisa Kane and Sanjay Swaminathan and Ming Wei Lin and Alberto Pinzon-Charry and Tiarni Templeton and Peter McNaughton and Thanh-Thao Adriana Le and Constance H. Katelaris and Julian Bosco and Lucinda J. Berglund},
  journal = {Frontiers in Immunology},
  year = {2026},
  month = oct,
  doi = {10.3389/fimmu.2026.1934715},
  url = {https://www.frontiersin.org/articles/10.3389/fimmu.2026.1934715/full},
  note = {CC BY 4.0},
}
RIS
TY  - JOUR
TI  - Enteropathy in inborn and acquired errors of immunity: histological patterns and clinical correlates
AU  - Meera Thangarajah
AU  - Samar Ojaimi
AU  - Alisa Kane
AU  - Sanjay Swaminathan
AU  - Ming Wei Lin
AU  - Alberto Pinzon-Charry
AU  - Tiarni Templeton
AU  - Peter McNaughton
AU  - Thanh-Thao Adriana Le
AU  - Constance H. Katelaris
AU  - Julian Bosco
AU  - Lucinda J. Berglund
PY  - 2026
DA  - 2026/10/15
JO  - Frontiers in Immunology
DO  - 10.3389/fimmu.2026.1934715
UR  - https://www.frontiersin.org/articles/10.3389/fimmu.2026.1934715/full
AB  - 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.  Methods: 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.  Results: 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).  Discussion: 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.
SN  - 1664-3224
ER  -