Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2012 Jul;171(7):1011-21.
doi: 10.1007/s00431-012-1714-8. Epub 2012 Mar 16.

Clinical practice : coeliac disease

C M Frank Kneepkens  1 , B Mary E von Blomberg
Affiliations
Review

Clinical practice : coeliac disease

C M Frank Kneepkens et al. Eur J Pediatr. 2012 Jul.

Abstract

Coeliac disease (CD) is an immune-mediated systemic condition elicited by gluten and related prolamines in genetically predisposed individuals and characterised by gluten-induced symptoms and signs, specific antibodies, a specific human leukocyte antigen (HLA) type and enteropathy. The risk of coeliac disease is increased in first-degree relatives, certain syndromes including Down syndrome and autoimmune disorders. It is thought to occur in 1 in 100-200 individuals, but still only one in four cases is diagnosed. Small-bowel biopsy is no longer deemed necessary in a subgroup of patients, i.e. when all of the following are present: typical symptoms or signs, high titres of and transglutaminase antibodies, endomysial antibodies, and HLA-type DQ2 or DQ8. In all other cases, small-bowel biopsy remains mandatory for a correct diagnosis. Therapy consists of a strictly gluten-free diet. This should result in complete disappearance of symptoms and of serological markers. Adequate follow-up is considered essential.

Conclusion: Although small-bowel biopsy may be omitted in a minority of patients, small-bowel biopsy is essential for a correct diagnosis of CD in all other cases. Diagnostic work-up should be completed before treatment with gluten-free diet instituted.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Marsh classification. From left to right: Marsh 0 = normal; 1 = increase of intraepithelial lymphocytes (IEL); 2 = increased IEL plus crypt hyperplasia; 3a = partial villous atrophy, 3b = subtotal villous atrophy, 3c = total villous atrophy
Fig. 2
Fig. 2
Algorithm for the diagnosis of coeliac disease in symptomatic children. TG2A transglutaminase type 2 antibodies, EmA endomysium antibodies, DGPA deamidated gliadin peptide antibodies, HLA human leukocyte antigen, N upper limit of normal, + present, − absent, Marsh Marsh classification
Fig. 3
Fig. 3
Algorithm for the diagnosis of coeliac disease in children with increased genetic risk and with associated immune disorders. TG2A transglutaminase type 2 antibodies, EmA endomysium antibodies, HLA human leukocyte antigen, N upper limit of normal, Marsh Marsh classification
Fig. 4
Fig. 4
Follow-up of coeliac disease
See this image and copyright information in PMC

References

    1. Akobeng AK, Thomas AG. Systematic review: tolerable amount of gluten for people with coeliac disease. Aliment Pharmacol Ther. 2008;27:1044–1052. doi: 10.1111/j.1365-2036.2008.03669.x. - DOI - PubMed
    1. Akobeng AK, Ramanan AV, Buchan I, Heller RF. Effect of breast feeding on risk of coeliac disease: a systematic review and meta-analysis of observational studies. Arch Dis Child. 2006;91:39–43. doi: 10.1136/adc.2005.082016. - DOI - PMC - PubMed
    1. Arentz-Hansen H, Korner R, Molberg O, et al. The intestinal T cell response to alpha-gliadin in adult celiac disease is focused on a single deamidated glutamine targeted by tissue transglutaminase. J Exp Med. 2000;191:603–612. doi: 10.1084/jem.191.4.603. - DOI - PMC - PubMed
    1. Arguelles‐Grande C, Tennyson CA, Lewis SK et al (2012) Variability in small bowel histopathology reporting between different pathology practice settings: Impact on the diagnosis of coeliac disease. J Clin Pathol 65:242–247 - PubMed
    1. Auricchio S, Greco L, Troncone R. Gluten-sensitive enteropathy in childhood. Pediatr Clin North Am. 1988;35:157–187. - PubMed