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

Abstract

ABSTRACT 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, human leukocyte antigen 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. Human leukocyte antigen 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.

Keywords

Affiliated Institutions

• Odense University Hospital DK
• Tel Aviv University IL
• Tampere University Hospital FI
• Hospital Universitari i Politècnic La Fe ES
• Schneider Children's Medical Center IL
• University of Debrecen HU
• Leiden University Medical Center NL
• University of Warmia and Mazury in Olsztyn PL
• Frederiksberg Hospital DK
• Carol Davila University of Medicine and Pharmacy RO
• Rijnstate Hospital NL
• Royal Hospital for Sick Children GB
• Ludwig-Maximilians-Universität München DE
• Tampere University FI

Related Publications