Coeliac disease: recognition, assessment and management

This guideline covers the recognition, assessment and management of coeliac disease in children, young people and adults.

# Recommendations
People have the right to be involved in discussions and make informed decisions about their care, as described in NICE's information on making decisions about your care.
Making decisions using NICE guidelines explains how we use words to show the strength (or certainty) of our recommendations, and has information about prescribing medicines (including off-label use), professional guidelines, standards and laws (including on consent and mental capacity), and safeguarding.

# Recognition of coeliac disease
Offer serological testing for coeliac disease to:
people with any of the following:
persistent unexplained abdominal or gastrointestinal symptoms
faltering growth
prolonged fatigue
unexpected weight loss
severe or persistent mouth ulcers
unexplained iron, vitamin B12 or folate deficiency
type 1 diabetes, at diagnosis
autoimmune thyroid disease, at diagnosis
irritable bowel syndrome (in adults)
firstdegree relatives of people with coeliac disease.
Consider serological testing for coeliac disease in people with any of the following:
metabolic bone disorder (reduced bone mineral density or osteomalacia)
unexplained neurological symptoms (particularly peripheral neuropathy or ataxia)
unexplained subfertility or recurrent miscarriage
persistently raised liver enzymes with unknown cause
dental enamel defects
Down's syndrome
Turner syndrome.
For people undergoing investigations for coeliac disease:
explain that any test is accurate only if a glutencontaining diet is eaten during the diagnostic process and
advise the person not to start a glutenfree diet until diagnosis is confirmed by a specialist, even if the results of a serological test are positive.
Advise people who are following a normal diet (containing gluten) to eat some gluten in more than 1 meal every day for at least 6 weeks before testing.
If people who have restricted their gluten intake or excluded gluten from their diet are reluctant or unable to reintroduce gluten into their diet before testing:
refer the person to a gastrointestinal specialist and
explain that it may be difficult to confirm their diagnosis by intestinal biopsy.
Advise people who have tested negative for coeliac disease, particularly firstdegree relatives and people with type 1 diabetes, that:
coeliac disease may present with a wide range of symptoms and
they should consult their healthcare professional if any of the symptoms listed in recommendations 1.1.1 or 1.1.2 arise or persist.
Do not offer serological testing for coeliac disease in infants before gluten has been introduced into the diet.

# Serological testing for coeliac disease
All serological tests should be undertaken in laboratories with clinical pathology accreditation (CPA) or ISO15189 accreditation.
When healthcare professionals request serological tests to investigate suspected coeliac disease in young people and adults, laboratories should:
test for total immunoglobulin A (IgA) and IgA tissue transglutaminase (tTG) as the first choice
use IgA endomysial antibodies (EMA) if IgA tTG is weakly positive
consider using IgG EMA, IgG deamidated gliadin peptide (DGP) or IgG tTG if IgA is deficient.
When healthcare professionals request serological tests to investigate suspected coeliac disease in children, laboratories should:
test for total IgA and IgA tTG as the first choice
consider using IgG EMA, IgG DGP or IgG tTG if IgA is deficient.
When laboratories test for total IgA, a specific assay designed to measure total IgA levels should be used.
Do not use human leukocyte antigen (HLA) DQ2 (DQ2.2 and DQ2.5)/DQ8 testing in the initial diagnosis of coeliac disease in nonspecialist settings.
Only consider using HLA DQ2 (DQ2.2 and DQ2.5)/DQ8 testing in the diagnosis of coeliac disease in specialist settings (for example, in children who are not having a biopsy, or in people who already have limited gluten ingestion and choose not to have a gluten challenge).
Laboratories should clearly communicate the interpretation of serological test results and recommended action to healthcare professionals.

# Referral of people with suspected coeliac disease
Refer young people and adults with positive serological test results to a gastrointestinal specialist for endoscopic intestinal biopsy to confirm or exclude coeliac disease. .
Refer children with positive serological test results to a paediatric gastroenterologist or paediatrician with a specialist interest in gastroenterology for further investigation for coeliac disease. .
Refer people with negative serological test results to a gastrointestinal specialist for further assessment if coeliac disease is still clinically suspected.
Healthcare professionals should have a low threshold for retesting people identified in recommendations 1.1.1 or 1.1.2 if they develop any symptoms consistent with coeliac disease.

# Monitoring in people with coeliac disease
Consider referring people with coeliac disease for endoscopic intestinal biopsy if continued exposure to gluten has been excluded and:
serological titres are persistently high and show little or no change after 12 months or
they have persistent symptoms, including diarrhoea, abdominal pain, weight loss, fatigue or unexplained anaemia.
Do not use serological testing alone to determine whether gluten has been excluded from the person's diet.
Offer an annual review to people with coeliac disease. During the review:
measure weight and height
review symptoms
consider the need for assessment of diet and adherence to the glutenfree diet
consider the need for specialist dietetic and nutritional advice.
Refer the person to a GP or consultant if concerns are raised in the annual review. The GP or consultant should assess all of the following:
the need for a dualenergy Xray absorptiometry (DEXA) scan (in line with the NICE guideline on osteoporosis: assessing the risk of fragility fracture) or active treatment of bone disease
the need for specific blood tests
the risk of longterm complications and comorbidities
the need for specialist referral.

# Nonresponsive and refractory coeliac disease
Consider the following actions in people with coeliac disease who have persistent symptoms despite advice to exclude gluten from their diet:
review the certainty of the original diagnosis
refer the person to a specialist dietitian to investigate continued exposure to gluten
investigate potential complications or coexisting conditions that may be causing persistent symptoms, such as irritable bowel syndrome, lactose intolerance, bacterial overgrowth, microscopic colitis or inflammatory colitis.
Diagnose refractory coeliac disease if the original diagnosis of coeliac disease has been confirmed, and exposure to gluten and any coexisting conditions have been excluded as the cause of continuing symptoms.
Refer people with refractory coeliac disease to a specialist centre for further investigation.
Consider prednisolone for the initial management of the symptoms of refractory coeliac disease in adults while waiting for specialist advice.

# Information and support
Explain to people who are thought to be at risk of coeliac disease that a delayed diagnosis, or undiagnosed coeliac disease, can result in continuing ill health and serious longterm complications.
Give people with coeliac disease (and their family members or carers, where appropriate) sources of information on the disease, including national and local specialist coeliac groups and dietitians with a specialist knowledge in coeliac disease.
A healthcare professional with a specialist knowledge of coeliac disease should tell people with a confirmed diagnosis of coeliac disease (and their family members or carers, where appropriate) about the importance of a glutenfree diet and give them information to help them follow it. This should include:
information on which types of food contain gluten and suitable alternatives, including glutenfree substitutes
explanations of food labelling
information sources about glutenfree diets, recipe ideas and cookbooks
how to manage social situations, eating out and travelling away from home, including travel abroad
avoiding cross contamination in the home and minimising the risk of accidental gluten intake when eating out
the role of national and local coeliac support groups.
Be aware that people with coeliac disease may experience anxiety and depression. Diagnose and manage these issues in line with the following NICE guidelines:
Depression in adults with a chronic physical health problem
Depression in children and young people
Generalised anxiety disorder and panic disorder (with or without agoraphobia) in adults
Social anxiety disorder.
Explain to people with coeliac disease and their family members or carers (as appropriate) that the Department of Health's Green Book recommends immunisation against pneumococcus.

# Advice on dietary management
Advise people with coeliac disease (and their family members or carers, where appropriate) to seek advice from a member of their healthcare team if they are thinking about taking overthecounter vitamin or mineral supplements.
Explain to people with coeliac disease (and their family members or carers, where appropriate) that they may need to take specific supplements such as calcium or vitamin D if their dietary intake is insufficient.
Explain to people with coeliac disease (and their family members or carers, where appropriate) that:
they can choose to include glutenfree oats in their diet at any stage and
they will be advised whether to continue eating glutenfree oats depending on their immunological, clinical or histological response.

# Terms used in this guideline

## IgA deficiency
IgA deficiency is defined as total IgA less than 0.07 g per litre.# Context
Coeliac disease is an autoimmune condition associated with chronic inflammation of the small intestine, which can lead to malabsorption of nutrients. Dietary proteins known as glutens, which are present in wheat, barley and rye, activate an abnormal mucosal immune response. Clinical and histological improvements usually follow when gluten is excluded from the diet.
Coeliac disease can present with a wide range of clinical features, both gastrointestinal (such as indigestion, diarrhoea, abdominal pain, bloating, distension or constipation) and nongastrointestinal (such as fatigue, dermatitis herpetiformis, anaemia, osteoporosis, reproductive problems, neuropathy, ataxia or delayed puberty). Children may also present with features such as faltering growth, static weight or progressive weight loss. Although some people present with typical symptoms, others will initially experience few or no symptoms.
Coeliac disease is a common condition. Population screening studies suggest that in the UK 1 in 100 people are affected. The complications of coeliac disease (which may or may not be present at diagnosis) can include osteoporosis, ulcerative jejunitis, malignancy (intestinal lymphoma), functional hyposplenism, vitamin D deficiency and iron deficiency.
People with conditions such as type 1 diabetes, autoimmune thyroid disease, Down's syndrome and Turner syndrome are at a higher risk than the general population of having coeliac disease. Firstdegree relatives of a person with coeliac disease also have an increased likelihood of having coeliac disease.
The treatment of coeliac disease is a lifelong glutenfree diet. Specific education and information, such as advice and education on alternative foods in the diet to maintain a healthy and varied intake, may increase the likelihood of adherence and a positive prognosis. These could be provided by a dietitian with experience in coeliac disease. However, access to specialist dietetic support is currently patchy in the UK.# Recommendations for research
The Guideline Committee has made the following recommendations for research. The Committee's full set of research recommendations is detailed in the full guideline.

# Serological testing in people who have IgA deficiency
What is the sensitivity and specificity of IgG tissue transglutaminase (tTG), IgG endomysial antibodies (EMA) and IgG deamidated gliadin peptide (DGP) tests in detecting coeliac disease in people with IgA deficiency?

## Why this is important
IgA deficiency is significantly more common in people with coeliac disease than in the general population. People with IgA deficiency will have a false negative result when tested for IgA antibody, which may lead to a missed diagnosis of coeliac disease. A missed diagnosis may result in increased use of NHS resources and the person experiencing the risks associated with undiagnosed coeliac disease. IgG antibodies are recommended for use in place of IgA antibodies in people who have IgA deficiency, but there is limited evidence to demonstrate the sensitivity and specificity of tests for IgG antibodies  that is, IgG tTG, IgG EMA and IgG DGP  in people suspected of having coeliac disease with IgA deficiency.

# Serological testing in people who test negative for antitransglutaminase
What is the sensitivity and specificity of IgA EMA and IgA DGP tests in detecting coeliac disease in people who test negative for IgA tTG?

## Why this is important
In people with suspected coeliac disease, IgA tTG is most commonly used as the firstchoice test to detect the presence of coeliac disease antibodies but some people with coeliac disease will get a false negative result. If this happens, and if there is a strong and ongoing clinical suspicion of coeliac disease, serological testing for IgA EMA or IgA or IgG DGP antibodies should also be requested. However, there is little evidence for the sensitivity and specificity of these antibodies in people who have tested negative for IgA tTG antibodies. A clearer understanding of the sensitivity and specificity of EMA and DGP antibodies in people who have tested negative for IgA tTG will allow clinicians to better interpret test results and make a more informed diagnosis.

# Dietary supplements
Should people with coeliac disease be offered calcium and vitamin D supplements for a specific time period soon after their initial diagnosis?

## Why this is important
People with coeliac disease are at an increased risk of malabsorption of key nutrients such as calcium and vitamin D. This is because of the role gluten plays in preventing these nutrients from being properly absorbed. It is not known how long the body takes to properly absorb these vitamins and minerals once a glutenfree diet is started. It is also not known whether the majority of people diagnosed with coeliac disease have enough calcium and vitamin D in their diet, or whether some people with coeliac disease are able to get enough of these nutrients from what they eat. Answering this research question will help healthcare professionals to understand whether calcium and vitamin D should be offered to everyone at the time of diagnosis and for how long these vitamin and mineral supplements should be taken.

# Dietitian contribution to patient management
How can the role of the dietitian contribute most effectively within a coeliac disease team?

## Why this is important
As a glutenfree diet is the primary treatment option for people with coeliac disease, it is important that a dietitian with a specialist interest in coeliac disease should play a significant role in their care and followup. Many of the common problems associated with the longterm management of coeliac disease happen because of nonadherence to a glutenfree diet. It is important to explore how to maximise the effectiveness of the dietitian's role in helping people with coeliac disease to adhere to a glutenfree diet.

# Frequency of monitoring
What is the effectiveness of more frequent monitoring compared with monitoring at 12 months after diagnosis in people with newly diagnosed coeliac disease?

## Why this is important
It is currently not known how often people with coeliac disease should have their condition monitored. No research has adequately investigated the effectiveness of different monitoring frequencies. There is variation across the UK in how often people with coeliac disease have their condition monitored. Further research within this area is important to ensure that people with coeliac disease are having their condition adequately monitored.