Increased production of interleukin-21, but not interleukin-17A, in the small intestine characterizes pediatric celiac disease

Mucosal Immunol. 2013 Nov;6(6):1202-13. doi: 10.1038/mi.2013.19. Epub 2013 Apr 10.

Abstract

Celiac disease (CD) is caused by inflammatory CD4(+) T-cell responses to dietary gluten. It is unclear whether interleukin (IL)-21 and IL-17A contribute to CD onset and lesion severity; therefore, we investigated IL-21 and IL-17A expression in biopsies from pediatric CD patients with different histopathological scores. High numbers of IL-21-producing cells were observed in pediatric CD lesions, even Marsh 1-2 lesions, whereas increased numbers of IL-17 secreting cells were not observed. Intraepithelial lymphocytes, CD4(+) T cells and also neutrophils secreted IL-21. Flow cytometry of lamina propria cells revealed a large population of IL-21- and interferon-γ (IFN-γ)-secreting CD3(+) T cells that did not secrete IL-17A. Adult CD patient biopsies also contained high numbers of IL-21-positive cells; however, enhanced numbers of IL-17-positive cells were observed in a small subgroup of patients with severe lesions. As duodenal tissue damage increases contact with microbe-associated molecular patterns, we hypothesized that microbial sensing by Toll-like receptors (TLRs) modulates T cell-derived cytokine secretion. Costimulation with TLR3 ligands during polyclonal T-cell activation significantly increased IL-21 secretion, whereas TLR2 ligands selectively enhanced IL-17A. These results demonstrate that an IL-17A-independent increase in IL-21 production by CD4(+) T cells is characteristic of pediatric CD. We hypothesize that incidental IL-17 secretion is caused by tissue damage rather than gluten-specific responses.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • CD4-Positive T-Lymphocytes / immunology*
  • Celiac Disease / immunology*
  • Cell Separation
  • Cells, Cultured
  • Child
  • Disease Progression
  • Flow Cytometry
  • Glutens / immunology
  • Humans
  • Interleukin-17 / genetics
  • Interleukin-17 / metabolism*
  • Interleukins / genetics
  • Interleukins / metabolism*
  • Intestine, Small / pathology
  • Lymphocyte Activation
  • Mucous Membrane / immunology*
  • Mucous Membrane / pathology
  • Toll-Like Receptor 2 / metabolism
  • Toll-Like Receptor 3 / metabolism
  • Up-Regulation

Substances

  • Interleukin-17
  • Interleukins
  • TLR2 protein, human
  • TLR3 protein, human
  • Toll-Like Receptor 2
  • Toll-Like Receptor 3
  • Glutens
  • interleukin-21