Evaluation of the ESPGHAN Celiac Guidelines in a North American Pediatric Population

Am J Gastroenterol. 2015 May;110(5):760-7. doi: 10.1038/ajg.2015.87. Epub 2015 Mar 31.

Abstract

Objectives: We retrospectively examined the performance of the tissue transglutaminase (TTG), endomysial antibody (EMA) tests, and the ESPGHAN (European Society of Paediatric Gastroenterology, Hepatology and Nutrition) nonbiopsy criteria in a pediatric population.

Methods: Consecutive celiac serologies and corresponding intestinal biopsy results were obtained on children <18 years old over 3.5 years. Patients were classified into three categories: positive TTG, negative TTG, and IgA deficiency.

Results: Of the 17,505 patients with celiac serology performed, 775 had a positive TTG, 574 with a negative TTG were biopsied, and 25 were IgA deficient. Of the patients with a TTG ≥10 × upper limit of normal (ULN), positive EMA, and symptoms, 98.2% had biopsies consistent with celiac disease (CD). Four human leukocyte antigen (HLA) DQ2/DQ8-positive patients who met the ESPGHAN nonbiopsy criteria did not have CD. In the group with a TTG 3-10 × ULN, 75.7% EMA-positive patients and only 40% EMA-negative patients had CD (P<0.001). Of those with a TTG 1-3 × ULN, 52.2% EMA-positive patients vs. only 13.3% EMA-negative patients had CD (P<0.01). Of the patients with bulbar and duodenal biopsies, 9.8% had CD confined only in the bulb, especially those with a low titer TTG (P<0.01). CD prevalence in our cohort was 34.6%. Sensitivity, specificity, and positive predictive value of the TTG were 98.7%, 86.4%, and 79.4%, respectively.

Conclusions: The TTG is a very sensitive screen for CD, but positive predictive value improves with a positive EMA titer. To apply the new ESPGHAN guidelines, clinicians must understand the performance of their celiac serology tests.

Publication types

  • Evaluation Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Autoantibodies / blood*
  • Biopsy
  • Celiac Disease / blood
  • Celiac Disease / diagnosis*
  • Celiac Disease / pathology
  • Child
  • Child, Preschool
  • Diet, Gluten-Free
  • Duodenum / pathology*
  • Female
  • GTP-Binding Proteins
  • HLA-DQ Antigens / blood
  • Humans
  • Immunoglobulin A / blood
  • Infant
  • Male
  • Practice Guidelines as Topic*
  • Predictive Value of Tests
  • Protein Glutamine gamma Glutamyltransferase 2
  • Retrospective Studies
  • Transglutaminases / blood*

Substances

  • Autoantibodies
  • HLA-DQ Antigens
  • HLA-DQ2 antigen
  • HLA-DQ8 antigen
  • Immunoglobulin A
  • Protein Glutamine gamma Glutamyltransferase 2
  • Transglutaminases
  • GTP-Binding Proteins