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European Society Paediatric Gastroenterology, Hepatology and Nutrition Guidelines for Diagnosing Coeliac Disease 2020

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  • Steffen Husby
  • Sibylle Koletzko
  • Ilma Korponay-Szabó
  • Kalle Kurppa
  • M Luisa Mearin
  • Carmen Ribes-Koninckx
  • Raanan Shamir
  • Riccardo Troncone
  • Renata Auricchio
  • Gemma Castillejo
  • Robin Christensen
  • Jernej Dolinsek
  • Peter Gillett
  • Asbjørn Hróbjartsson
  • Tunde Koltai
  • Markku Maki
  • Sabrina Mai Nielsen
  • Alina Popp
  • Ketil Størdal
  • Katharina Werkstetter
  • Margreet Wessels
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OBJECTIVES: The ESPGHAN 2012 coeliac disease (CD) diagnostic guidelines aimed to guide physicians in accurately diagnosing CD and permit omission of duodenal biopsies in selected cases. Here, an updated and expanded evidence-based guideline is presented.

METHODS: Literature databases and other sources of information were searched for studies that could inform on 10 formulated questions on symptoms, serology, HLA genetics, and histopathology. Eligible articles were assessed using QUADAS2. GRADE provided a basis for statements and recommendations.

RESULTS: Various symptoms are suggested for case finding, with limited contribution to diagnostic accuracy. If CD is suspected, measurement of total serum IgA and IgA-antibodies against transglutaminase 2 (TGA-IgA) is superior to other combinations. We recommend against deamidated gliadin peptide antibodies (DGP-IgG/IgA) for initial testing. Only if total IgA is low/undetectable, an IgG-based test is indicated. Patients with positive results should be referred to a paediatric gastroenterologist/specialist. If TGA-IgA is ≥10 times the upper limit of normal (10× ULN) and the family agrees, the no-biopsy diagnosis may be applied, provided endomysial antibodies (EMA-IgA) will test positive in a second blood sample. HLA DQ2-/DQ8 determination and symptoms are not obligatory criteria. In children with positive TGA-IgA <10× ULN at least 4 biopsies from the distal duodenum and at least 1 from the bulb should be taken. Discordant results between TGA-IgA and histopathology may require re-evaluation of biopsies. Patients with no/mild histological changes (Marsh 0/I) but confirmed autoimmunity (TGA-IgA/EMA-IgA+) should be followed closely.

CONCLUSIONS: CD diagnosis can be accurately established with or without duodenal biopsies if given recommendations are followed.

Original languageEnglish
JournalJournal of Pediatric Gastroenterology and Nutrition
Volume70
Issue number1
Pages (from-to)141-156
Number of pages16
ISSN0277-2116
DOIs
Publication statusPublished - Jan 2020

ID: 60007627