Celiac disease: from oral tolerance to intestinal inflammation, autoimmunity and lymphomagenesis

Mucosal Immunol. 2009 Jan;2(1):8-23. doi: 10.1038/mi.2008.75. Epub 2008 Oct 29.

Abstract

Celiac disease is a multifactorial disorder and provides a privileged model to decipher how the interplay between environmental and genetic factors can alter mucosal tolerance to a food antigen, lead to chronic intestinal inflammation, and ultimately promote T-cell lymphomagenesis. Here we summarize how HLA-DQ2/8 molecules, the main genetic risk factor for this disease can orchestrate a CD4(+) T-cell adaptive immune response against gluten, and discuss recent data which shed light on the innate and adaptive immune stimuli that collaborate to induce a proinflammatory TH1 response, a massive expansion of intraepithelial lymphocytes, and a cytolytic attack of the epithelium. The intestinal immune response driven in genetically predisposed patients by chronic exposure to gluten emerges as the pathological counterpart of normal acute intestinal responses to intracellular pathogens.

Publication types

  • Comment
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Autoimmunity / immunology*
  • Celiac Disease / complications*
  • Celiac Disease / immunology*
  • Humans
  • Immune Tolerance / immunology*
  • Inflammatory Bowel Diseases / complications
  • Inflammatory Bowel Diseases / immunology*
  • Lymphoma / complications
  • Lymphoma / immunology*
  • Mouth Mucosa / immunology*