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European Society for Pediatric Gastroenterology, Hepatology, and Nutrition Guidelines for the Diagnosis of Coeliac Disease

Husby, S.*; Koletzko, S.; Korponay-Szabó, I.R.; Mearin, M.L.§; Phillips, A.||; Shamir, R.; Troncone, R.#; Giersiepen, K.**; Branski, D.††; Catassi, C.‡‡; Lelgeman, M.§§; Mäki, M.||||; Ribes-Koninckx, C.¶¶; Ventura, A.##; Zimmer, K.P.****; for the ESPGHAN Working Group on Coeliac Disease Diagnosis, on behalf of the ESPGHAN Gastroenterology Committee


In the article that appeared on page 136 of the January 2012 issue, a number of errors are noted, as follows:

* Page 138, paragraph 4, the sentence should read: When duodenal biopsies, taken during routine diagnostic workup for gastrointestinal symptoms, disclose a histological pattern indicative of CD (Marsh 1–3 lesions), antibody determinations and HLA typing should be performed.

* Page 139, last paragraph, the sentence should read: In 2008, the UK National Institute for Health and Clinical Evidence (NICE) published guidelines for the diagnosis and management of CD in general practice (78a).

* Page 140, under “Symptoms and Signs,” the sentence should read: Gastrointestinal symptoms frequently appear in clinically diagnosed childhood CD, including diarrhoea in about 50% of patients (15,16).

* Page 144, Table 3, the second sentence in the footnote should read: Data also from Richtlijn Coeliakie en Dermatitis Herpetiformis. Kwaliteitsinstituut voor de Gesondheidszorg CBO (33a).

* Page 144, line 10, the sentence should read:The sensitivity of HLA-DQ2 is high (median 91%; p25 – p7586.3%– 94.0%), and if combined with HLA-DQ8 (at least 1 is positive), it is even higher (median 96.2%; p25 – p75 94.6%–99.8%), making extremely small the chance of an individual who is negative for DQ2 and DQ8 to have CD; even though the small percentage of HLA-DQ2-negative and HLA-DQ8-negative patients is well documented (33–35).

* Page 144, Table 4, the second sentence in the footnote should read: Data also from Richtlijn Coeliakie en Dermatitis Herpetiformis. Kwaliteitsinstituut voor de Gesondheidszorg CBO (33a).

* Page 146, paragraph 6, the penultimate line should read: Anti-TG2 antibodies also can be detected in saliva. Sufficient sensitivity and specificity was not achieved with conventional commercially available immunoassays (63), although the use of radiobinding assays appeared to be more favourable (64).

* Page 146, paragraph 8, the first sentence should read: The positivity for anti-TG2 and/or EMA is associated with a high probability for CD in children and adolescents (10,52); however, low levels of anti-TG2 have been described in a number of conditions unrelated to CD, such as other autoimmune diseases, infections, tumours, myocardial damage, liver disorders, and psoriasis (62,68 – 70).

* Page 147, statement 3.3.5, the second sentence should read: For other tests, values considered to be high antibody positivity should be established by comparison with a panel of tests, which are listed in Appendix I.

* Page 147, statement 3.3.8, the first sentence should read: High concentrations of anti-TG2 antibodies in blood (as defined in statement 3.3.5) predict villous atrophy better than low positive or borderline values.

* Page 149, statement 3.4.10, the voting results should read: Total number of votes: 13, Agree: 12, Disagree: 1, Abstentions: 0

* Page 159, reference 78a should read: National Institute for Health and Clinical Excellence. CG86 coeliac disease: full guideline. NICE guidelines. Accessed August 31, 2011.

Figures 1 and 2 are replaced as follows:

Journal of Pediatric Gastroenterology and Nutrition. 54(4):572-573, April 2012.

Journal of Pediatric Gastroenterology & Nutrition: January 2012 - Volume 54 - Issue 1 - p 136–160
doi: 10.1097/MPG.0b013e31821a23d0
Clinical Guidelines

Objective: Diagnostic criteria for coeliac disease (CD) from the European Society for Paediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) were published in 1990. Since then, the autoantigen in CD, tissue transglutaminase, has been identified; the perception of CD has changed from that of a rather uncommon enteropathy to a common multiorgan disease strongly dependent on the haplotypes human leukocyte antigen (HLA)-DQ2 and HLA-DQ8; and CD-specific antibody tests have improved.

Methods: A panel of 17 experts defined CD and developed new diagnostic criteria based on the Delphi process. Two groups of patients were defined with different diagnostic approaches to diagnose CD: children with symptoms suggestive of CD (group 1) and asymptomatic children at increased risk for CD (group 2). The 2004 National Institutes of Health/Agency for Healthcare Research and Quality report and a systematic literature search on antibody tests for CD in paediatric patients covering the years 2004 to 2009 was the basis for the evidence-based recommendations on CD-specific antibody testing.

Results: In group 1, the diagnosis of CD is based on symptoms, positive serology, and histology that is consistent with CD. If immunoglobulin A anti-tissue transglutaminase type 2 antibody titers are high (>10 times the upper limit of normal), then the option is to diagnose CD without duodenal biopsies by applying a strict protocol with further laboratory tests. In group 2, the diagnosis of CD is based on positive serology and histology. HLA-DQ2 and HLA-DQ8 testing is valuable because CD is unlikely if both haplotypes are negative.

Conclusions: The aim of the new guidelines was to achieve a high diagnostic accuracy and to reduce the burden for patients and their families. The performance of these guidelines in clinical practice should be evaluated prospectively.

*Hans Christian Andersen Children's Hospital at Odense University Hospital

Division of Paediatric Gastroenterology and Hepatology, Dr. von Hauner Children's Hospital, Ludwig-Maximilians-University

University of Debrecen, Medical and Health Science Center

§Department of Paediatrics, Leiden University Medical Center

||University College London Medical School/Paediatrics and Child Health

Institute of Gastroenterology, Nutrition and Liver Diseases, Schneider Children's Medical Center of Israel, Sackler Faculty of Medicine, Tel-Aviv University

#Department of Paediatrics and European Laboratory for the Investigation of Food-Induced Diseases, University “Federico II”

**Centre for Social Policy Research, University of Bremen

††Department of Paediatrics, Hadash University Hospitals

‡‡Department of Paediatrics, Università Politecnica delle Marche

§§Medical Review Board of the Statutory Health Insurance Fund

||||Paediatric Research Centre, University of Tampere and Tampere University Hospital

¶¶La Fe University Hospital

##Department of Paediatrics, IRCCS Burlo Garofolo University of Trieste

****Department for General Paediatrics and Neonatology, Justus-Liebig University.

Address correspondence and reprint requests to Dr Steffen Husby (e-mail:

Received 1 September, 2011

Accepted 1 September, 2011

Drs Husby, Koletzko, Korponay-Szabó, Mearin, Phillips, Shamir, Troncone, and Giersiepen contributed equally to the article and are listed as first authors.

Conflict of interest statements are listed at the end of the article.

Copyright 2012 by ESPGHAN and NASPGHAN