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Practice Guidelines

Recognition and assessment of coeliac disease in children and adults: summary of NICE guidance

BMJ 2009; 338 doi: https://doi.org/10.1136/bmj.b1684 (Published 27 May 2009) Cite this as: BMJ 2009;338:b1684
  1. Roberta Richey, technical analyst1,
  2. Peter Howdle, professor of clinical medicine2,
  3. Elizabeth Shaw, technical adviser1,
  4. Tim Stokes, associate director1
  5. on behalf of the Guideline Development Group
  1. 1Centre for Clinical Practice (Short Clinical Guidelines), National Institute for Health and Clinical Excellence, Manchester M1 4BD
  2. 2Leeds Institute of Molecular Medicine, St James’s University Hospital, Leeds LS9 7TF
  1. Correspondence to: P Howdle p.d.howdle{at}leeds.ac.uk

    Why read this summary?

    Coeliac disease is an autoimmune condition that can be diagnosed at any age. Although it has been traditionally associated with mainly gastrointestinal signs and symptoms, its non-gastrointestinal features have been increasingly recognised.

    Given its varied clinical manifestations and the historical belief that it is relatively uncommon,1 2 concern has been raised that coeliac disease—and its possible long term consequences—is being underdiagnosed. It has also been shown to be more prevalent in people with other autoimmune conditions.3 This article summarises the most recent recommendations from the National Institute for Health and Clinical Excellence (NICE) on the recognition and assessment of coeliac disease.4

    Recommendations

    NICE recommendations are based on systematic reviews of best available evidence. When minimal evidence is available, recommendations are based on the Guideline Development Group’s experience and opinion of what constitutes good practice. Evidence levels for the recommendations are given in italic in square brackets.

    Who should be offered serological testing for coeliac disease?

    • Offer testing to children and adults with any of the following signs and symptoms:

      • -Chronic or intermittent diarrhoea

      • -Failure to thrive or faltering growth (in children)

      • -Persistent and unexplained gastrointestinal symptoms including nausea or vomiting

      • -Prolonged fatigue

      • -Recurrent abdominal pain, cramping, or distension

      • -Sudden or unexpected weight loss

      • -Unexplained iron deficiency anaemia or other unspecified anaemia.

    [Based on moderate quality evidence from cohort studies]

    • Offer testing to children and adults with any of the following conditions:

      • -Autoimmune thyroid disease

      • -Dermatitis herpetiformis

      • -Irritable bowel syndrome

      • -Type 1 diabetes.

    • Offer testing to children and adults who have first degree relatives with coeliac disease.

    [Based on moderate quality evidence from cohort studies]

    • Consider offering testing to children or adults with any of the following:

      • -Addison’s disease

      • -Amenorrhoea

      • -Aphthous stomatitis

      • -Autoimmune liver conditions

      • -Autoimmune myocarditis

      • -Chronic thrombocytopenia purpura

      • -Dental enamel defects

      • -Depression or bipolar disorder

      • -Down’s syndrome

      • -Epilepsy

      • -Low trauma fracture

      • -Lymphoma

      • -Metabolic bone disease (such as rickets or osteomalacia)

      • -Microscopic colitis

      • -Persistent or unexplained constipation

      • -Persistently raised liver enzymes with unknown cause

      • -Polyneuropathy

      • -Recurrent miscarriage

      • -Reduced bone mineral density

      • -Sarcoidosis

      • -Sjögren’s syndrome

      • -Turner’s syndrome

      • -Unexplained alopecia

      • -Unexplained subfertility.

    [Based on moderate quality evidence from cohort studies and case control studies and the experience and opinion of the Guideline Development Group (GDG)]

    • Do not use serological testing for coeliac disease in infants before gluten has been introduced into the diet. [Based on the experience and opinion of the GDG]

    Advice to patients

    • Inform people that testing for coeliac disease is accurate only if the person is eating a diet that contains gluten at the time of testing.

    • Inform people that when following a normal gluten containing diet they should eat some gluten (for example, bread, chapattis, pasta, biscuits, or cakes) in more than one meal every day for a minimum of six weeks before testing.

    • If a person is reluctant or unable to reintroduce gluten into their diet before testing refer them to a gastrointestinal specialist and inform them that it may be difficult to confirm a diagnosis of coeliac disease on intestinal biopsy.

    • Inform people and their parents or carers that a delayed diagnosis of coeliac disease, or undiagnosed coeliac disease, can result in:

      • -Continuing ill health

      • -Long term complications, including osteoporosis and increased risk of fracture, unfavourable pregnancy outcomes, and a modest increased risk of intestinal malignancy

      • -In children, growth failure, delayed puberty, and dental problems.

      • [Based on moderate quality evidence from observational studies and on the experience and opinion of the GDG]

    Serological tests

    • All tests should be undertaken in laboratories with clinical pathology accreditation. [Based on the experience and opinion of the GDG]

    • Do not use IgG or IgA antigliadin antibody (AGA) tests in the diagnosis of coeliac disease. [Based on good quality evidence from cohort studies and health economic evidence]

    • When clinicians request serology, laboratories should:

      • -Use IgA tissue transglutaminase antibody (tTGA) testing as the first choice of test

      • -Use IgA endomysial antibody (EMA) testing if the result of the IgA tissue transglutaminase antibody test is equivocal

      • -Check for IgA deficiency if serology is negative (if the laboratory detects a low optical density on the IgA tTGA test, very low IgA tTGA results, or low background on the IgA EMA test)

      • -Use IgG tissue transglutaminase antibody or IgG endomysial antibody tests (or both) for people with confirmed IgA deficiency

      • Communicate clearly the results in terms of values, interpretation, and recommended action.

    [Based on good quality evidence from cohort studies and on a health economic analysis]

    Referral for intestinal biopsy

    • Offer anybody with positive serological results a referral to a gastrointestinal specialist for intestinal biopsy to confirm or exclude coeliac disease.

    • If serology tests are negative but coeliac disease is still clinically suspected, offer referral to a gastrointestinal specialist for further assessment.

    [Based on the experience and opinion of the GDG]

    Overcoming barriers

    Coeliac disease was previously thought to be uncommon, and the best serological tests for diagnosing this condition are unclear. This guideline emphasises the variety of symptoms and signs that can arise from coeliac disease and the many conditions that may coexist with it. It aims to raise awareness of coeliac disease and increase appropriate diagnosis, thus enabling effective treatment and improvement of the health and quality of life of people with the disease.

    Further information on the guidance

    Methods

    This guideline was developed as a short clinical guideline and followed the current process for the development of NICE short clinical guidelines outlined in the NICE guidelines manual (www.nice.org.uk). The Guideline Development Group then discussed the evidence and drew up recommendations. A health economic model was also developed to determine the cost effectiveness of serological tests for coeliac disease.

    The guideline went through an external consultation with stakeholders. The development group then assessed stakeholders’ comments and appropriately modified the guideline.

    NICE has produced three different versions of the guideline: a full version, a quick reference guide, and a version for patients and the public. All versions are available from the NICE website.

    Areas of future research
    Repeat serological testing

    Should serological tests for coeliac disease be repeated, and if so how often? Serological tests give low rates of false negative results, but it is not clear whether coeliac disease develops over time—that is, can a person be truly negative at one point in time and later be truly positive? Evidence on the need for repeat testing is lacking.

    Dietary gluten and accuracy of tests

    What is the minimum gluten dietary content necessary for the optimal accuracy of serological tests and intestinal biopsy for the diagnosis of coeliac disease? No robust evidence is available on how much gluten people should include in their diet when being tested for coeliac disease.

    Notes

    Cite this as: BMJ 2009;338:b1684

    Footnotes

    • This is one of a series of BMJ summaries of new guidelines, which are based on the best available evidence; they highlight important recommendations for clinical practice, especially where uncertainty or controversy exists.

    • Guideline development group: Mohamed Abuzakouk, Sorrel Burden, Peter Howdle (chair), Alison Lister, Peter Macfarlane, Norma McGough, John O’Malley, David Saunders, Adrian Thomas, Julian Walters, and David Wray. NICE Short Guidelines Technical Team: Michael Heath, Ruth McAllister, Fergus Macbeth, Roberta Richey, Elizabeth Shaw, Tim Stokes, and Daniel Tuvey.

    • Contributors: RR drafted the summary; ES, TS, and PH reviewed the contents. All authors approved the final version. TS is the guarantor.

    • Funding: This summary was written by the Centre for Clinical Practice (Short Clinical Guidelines Technical Team) at the National Institute for Health and Clinical Excellence.

    • Competing interests: None declared.

    • Provenance and peer review: Commissioned; not externally peer reviewed.

    References

    View Abstract