ArticleClinical and translational gastroenterology2016
Uncontrolled IL-17 Production by Intraepithelial Lymphocytes in a Case of non-IPEX Autoimmune Enteropathy.
Article in Clinical and translational gastroenterology, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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Who cites it
10 citing papers in PubMed, 17 citations in OpenAlex.
- Pro-inflammatory intestinal Th17-cells are tissue-resident, accumulate in the epithelia in Crohn's disease, and predict unresponsiveness to vedolizumab.Journal of Crohn's & colitis · 2026Article
- Mucosal microbiota and intraepithelial lymphocyte remodeling link maternal immune activation to neuroimmune outcomes in female and male offspring.Frontiers in immunology · 2026Article
- Comprehensive insights into pathogenesis, diagnosis, treatment, and prognosis in adult autoimmune enteropathy.Orphanet journal of rare diseases · 2025Review
- Evaluation of the immune response of peripheral blood mononuclear cells cultured on Ti6Al4V-ELI polished or etched surfaces.Frontiers in bioengineering and biotechnology · 2024Article
- An Intestinal Th17 Subset is Associated with Inflammation in Crohn's Disease and Activated by Adherent-invasive Escherichia coli.Journal of Crohn's & colitis · 2023Article
- Characterization of Bovine Intraepithelial T Lymphocytes in the Gut.Pathogens (Basel, Switzerland) · 2023Article
- Acute Flare of Adult-Onset Autoimmune Enteropathy Associated With Cyclophosphamide.ACG case reports journal · 2021Article
- [Autoimmune enteropathy in adults : A rare and difficult but relevant differential diagnosis of chronic diarrhea].Der Pathologe · 2020Article
- Association of Serum T cell Immunoglobulin Domain and Mucin-3 and Interleukin-17 with Systemic Lupus Erythematosus.Medical science monitor basic research · 2018Article
- Gammadelta T Cells: Unconventional T Cells Involved in IBD Pathogenesis.Digestive diseases and sciences · 2018Article
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Authors and funding
13 authors at 3 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectivesTo provide a functional and phenotypic characterization of immune cells infiltrating small intestinal mucosa during non-IPEX autoimmune enteropathy (AIE), as to gain insights on the pathogenesis of this clinical condition.
methodsDuodenal biopsies from a patient with AIE at baseline and following drug-induced remission were analyzed by immunohistochemistry, immunofluorescence, and flow cytometry, and results were compared with those obtained from patients with active celiac disease, ileal Crohn's disease and healthy controls. Lamina propria (LP) and intraepithelial (IELs) lymphocytes from AIE and controls were analyzed for mechanisms regulating cytokine production. Foxp3 expression and suppressive functions of LP regulatory T cells (Tregs) were analyzed.
resultsThe quantitative deficit of Foxp3 expression in Tregs in AIE associates with unrestrained IL-17 production by IELs. Interleukin (IL)-17-producing IELs were rare in the uninflamed duodenum and in the ileum of Crohn's disease patients, and disappeared upon drug-induced AIE remission. IL-17 upregulation in CD4(+)IELs and CD4(+)LP T cells had different requirements for pro-inflammatory cytokines. Moreover, transforming growth factor-β (TGF-β) selectively enhanced IL-17 production by CD8(+)IELs. Intriguingly, although Foxp3(low)Tregs in AIE were poorly suppressive, they could upregulate GARP-LAP/TGF-β surface expression and enhanced IL-17 production selectively by CD8(+)IELs. Finally, phosphorylated Smad2/3 was detectable in duodenal CD8(+) lymphocytes in active AIE in situ, indicating that they received signals from the TGF-β receptor in vivo.
conclusionsAIE is characterized by the appearance of unconventional IL-17-producing IELs, which could be generated locally by pro-inflammatory cytokines and TGF-β. These results suggest that Foxp3(+)Tregs and Treg-derived TGF-β regulate IL-17 production by IELs in the small intestine and in AIE.
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