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Management of celiac disease in daily clinical practice

  • Author Footnotes
    1 Luca Elli and Francesca Ferretti equally participated to the manuscript preparation
    Luca Elli
    Correspondence
    Corresponding author at: Center for Prevention and Diagnosis of Celiac Disease, Fondazione IRCCS Ca'Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, 20122 Milano, Italy.
    Footnotes
    1 Luca Elli and Francesca Ferretti equally participated to the manuscript preparation
    Affiliations
    Center for Prevention and Diagnosis of Celiac Disease, Division of Gastroenterology and Endoscopy, Fondazione IRCCS Ca'Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, 20122 Milano, Italy
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  • Author Footnotes
    1 Luca Elli and Francesca Ferretti equally participated to the manuscript preparation
    Francesca Ferretti
    Footnotes
    1 Luca Elli and Francesca Ferretti equally participated to the manuscript preparation
    Affiliations
    Center for Prevention and Diagnosis of Celiac Disease, Division of Gastroenterology and Endoscopy, Fondazione IRCCS Ca'Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, 20122 Milano, Italy

    Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Via Festa del Perdono, 20122 Milano, Italy
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  • Stefania Orlando
    Affiliations
    Center for Prevention and Diagnosis of Celiac Disease, Division of Gastroenterology and Endoscopy, Fondazione IRCCS Ca'Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, 20122 Milano, Italy
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  • Maurizio Vecchi
    Affiliations
    Center for Prevention and Diagnosis of Celiac Disease, Division of Gastroenterology and Endoscopy, Fondazione IRCCS Ca'Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, 20122 Milano, Italy

    Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Via Festa del Perdono, 20122 Milano, Italy
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  • Erika Monguzzi
    Affiliations
    Center for Prevention and Diagnosis of Celiac Disease, Division of Gastroenterology and Endoscopy, Fondazione IRCCS Ca'Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, 20122 Milano, Italy

    Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Via Festa del Perdono, 20122 Milano, Italy

    Institute for Translational Immunology, Research Center for Immunotherapy (FZI), Johannes Gutenberg University (JGU) Medical Center, 55101 Mainz, Germany
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  • Leda Roncoroni
    Affiliations
    Center for Prevention and Diagnosis of Celiac Disease, Division of Gastroenterology and Endoscopy, Fondazione IRCCS Ca'Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, 20122 Milano, Italy

    Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Via Festa del Perdono, 20122 Milano, Italy

    Department of Biomedical, Surgical and Dental Sciences, Università degli Studi di Milano, Via Festa del Perdono, 20122 Milano, Italy
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  • Detlef Schuppan
    Affiliations
    Institute for Translational Immunology, Research Center for Immunotherapy (FZI), Johannes Gutenberg University (JGU) Medical Center, 55101 Mainz, Germany

    Division of Gastroenterology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02215, USA
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  • Author Footnotes
    1 Luca Elli and Francesca Ferretti equally participated to the manuscript preparation
Published:December 05, 2018DOI:https://doi.org/10.1016/j.ejim.2018.11.012

      Highlights

      • Celiac disease is the most common severe enteropathy in Western countries
      • Gluten is the dietary trigger of CD and the autoantigen tissue transglutaminase is involved in the immune-mediated pathogenesis,
      • Anti-transglutaminase antibodies and duodenal biopsy are cornerstones of diagnosis
      • Missed and incorrect diagnoses continue to be a clinical problem
      • A strict lifelong gluten-free diet is required for resolution and stable remission

      Abstract

      Celiac disease (CD) is the most common autoimmune enteropathy worldwide. In CD, dietary gluten triggers a T cell driven small intestinal inflammation in a subset of genetically predisposed subjects, expressing the HLA DQ2 and/or DQ8 genes on their antigen presenting cells. HLA DQ2/DQ8 can bind gluten peptides after their prior modification by the CD autoantigen, tissue transglutaminase (TG2). This process leads to the activation of gluten reactive T cells, small bowel villous atrophy, crypt hyperplasia and intraepithelial lymphocytosis, the histological hallmarks of CD. The clinical picture of CD is extremely heterogeneous including intestinal (especially diarrhea, abdominal pain, bloating) and extraintestinal (especially associated autoimmune diseases, anemia, osteoporosis) manifestations. The prevalence of CD in most parts of the world is estimated at 1:100–1:150 and its diagnosis is based on the presence of circulating autoantibodies (anti-TG2) and the histological detection of villous atrophy. Treatment is a lifelong gluten free diet but adjunctive therapies are in development. Although CD is a well-characterized disease, it is grossly underdiagnosed, despite the severe consequences of long-term gluten ingestion in CD, such as enhanced autoimmunity, refractory CD and intestinal T cell lymphoma. The aim of the presented review is to provide a clinical guide and to summarize the most recent clinical progress in CD research.

      Keywords

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