Immune-related enteropathy

Curr Opin Gastroenterol. 2026 May 1;42(3):189-200. doi: 10.1097/MOG.0000000000001162. Epub 2026 Mar 5.

Abstract

Purpose of review: Immune-related enteropathies (IREs) represent a heterogeneous group of disorders characterised by immune-mediated small bowel injury. While coeliac disease is the most common cause, widespread use of small bowel endoscopy has expanded the recognition of rarer causes. This review aims to summarise recent advances in diagnosis, classification and management of IRE.

Recent findings: Significant overlap exists between coeliac disease, autoimmune enteropathy, collagenous sprue, drug-induced enteropathies, common variable immunodeficiency (CVID) associated enteropathy and other IRE. A recent meta-analysis supports a no-biopsy approach in selected adults with significantly elevated IgA tissue transglutaminase levels and suspected coeliac disease. Refractory coeliac disease type II is now recognised as a low-grade intraepithelial T-cell lymphoma with a high risk of progression to enteropathy-associated T-cell lymphoma (EATL). Drug-induced enteropathies, particularly olmesartan-associated enteropathy, are increasingly detected and may mimic autoimmune enteropathy histologically. Novel therapeutic approaches, including cladribine, JAK inhibitors and biologics, have shown promise in refractory disease, although evidence is limited to small studies.

Summary: IREs present substantial diagnostic and therapeutic challenges due to overlapping characteristics and variable disease course. A structured approach integrating clinical, serological and histopathological data is essential for accurate diagnosis. Early recognition and tailored management are crucial to prevent long-term complications.

Keywords: immune-mediated enteropathy; immune-related enteropathy; small bowel.

Publication types

  • Review

MeSH terms

  • Autoimmune Diseases / diagnosis
  • Autoimmune Diseases / immunology
  • Celiac Disease / diagnosis
  • Celiac Disease / immunology
  • Celiac Disease / therapy
  • Humans
  • Intestinal Diseases* / diagnosis
  • Intestinal Diseases* / immunology
  • Intestinal Diseases* / therapy
  • Intestine, Small / immunology
  • Intestine, Small / pathology
  • Polyendocrinopathies, Autoimmune

Supplementary concepts

  • Autoimmune enteropathy