Steffen Husby1, Sibylle Koletzko2, Ilma Korponay-Szabó3, Kalle Kurppa4, Maria Luisa Mearin5, Carmen Ribes-Koninckx6, Raanan Shamir7, Riccardo Troncone8, Renata Auricchio8, Gemma Castillejo9, Robin Christensen10, Jernej Dolinsek11, Peter Gillett12, Asbjørn Hróbjartsson13, Tunde Koltai14, Markku Maki4, Sabrina Mai Nielsen10, Alina Popp15, Ketil Størdal16, Katharina Werkstetter2, Margreet Wessels17. 1. Hans Christian Andersen Children's Hospital, Odense University Hospital, DK-5000 Odense C, Denmark. 2. Department of Pediatrics, Dr. von Hauner Children's Hospital, University Hospital, LMU Munich, Germany and Department of Pediatrics, Gastroenterology and Nutrition, School of Medicine Collegium Medicum University of Warmia and Mazury, Olsztyn, Poland. 3. Heim Pál National Paediatric Institute, Coeliac Disease Centre, Budapest, and Department of Paediatrics, University of Debrecen Medical Faculty, Debrecen, Hungary. 4. Tampere Centre for Child Health Research, Tampere University, and Department of Pediatrics, Tampere University Hospital, Tampere, Finland. 5. Department of Pediatrics, Leiden University Medical Center, Leiden, The Netherlands. 6. Pediatric Gastroenterology Unit. La Fe University Hospital. Valencia. Spain. 7. Institute for Gastroenterology, Nutrition and Liver Diseases, Schneider Children's Medical Center, Petach Tikva, Sackler Faculty of Medicine, Tel Aviv University, Israel. 8. Department of Medical Translational Sciences and European Laboratory for the Investigation of Food-Induced Diseases, University Federico II, Naples, Italy. 9. Pediatric Gastroenterology Unit, Department of Pediatrics, Hospital Universitari Sant Joan de Reus, Reus, Spain. 10. Musculoskeletal Statistics Unit: The Parker Institute, Bispebjerg and Frederiksberg Hospital & Department of Rheumatology, Odense University Hospital, Denmark. 11. Unit of Pediatric Gastroenterology and Nutrition, University Medical Centre Maribor, Maribor, Slovenia. 12. Paediatric Gastroenterology, Hepatology and Nutrition Department, Royal Hospital for Sick Children, Edinburgh, Scotland, UK. 13. Centre for Evidence Based Medicine Odense (CEBMO), Odense University Hospital, Denmark. 14. Association of European Coeliac Society/Belgium, Hungarian Coeliac Society/Hungary. 15. University of Medicine and Pharmacy "Carol Davila", National Institute for Mother and Child Health, Bucharest, Romania. 16. Norwegian Institute of Public Health, Oslo and Ostfold Hospital Trust, Norway. 17. Department of Pediatrics, Rijnstate Hospital, Arnhem, the Netherlands.
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, 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.
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.
Authors: Giulio Maltoni; Maximiliano Zioutas; Marta Mosticchio; Lorenzo Iughetti; Barbara Predieri; Patrizia Bruzzi; Brunella Iovane; Pietro Lazzeroni; Vanna Graziani; Tosca Suprani; Sara Monti; Maria E Street; Anna Lasagni; Francesca De Luca; Francesca Libertucci; Benedetta Mainetti; Sara Riboni; Paola Sogno Valin; Andrea Pession; Stefano Zucchini Journal: Eur J Pediatr Date: 2022-01-14 Impact factor: 3.183
Authors: Govind K Makharia; Prashant Singh; Carlo Catassi; David S Sanders; Daniel Leffler; Raja Affendi Raja Ali; Julio C Bai Journal: Nat Rev Gastroenterol Hepatol Date: 2022-01-03 Impact factor: 46.802