1.1. Introduction

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 non-gastrointestinal (such as fatigue, dermatitis herpetiformis, anaemia, osteoporosis, reproductive problems, short stature, neuropathy, ataxia or delayed puberty). Although some people present with typical symptoms, others have few or no symptoms.

People with autoimmune conditions such as type 1 diabetes and autoimmune thyroid disease, or people with a first-degree family history of coeliac disease, have an increased likelihood of coeliac disease.

1.2. Health and resource burden

Coeliac disease is a common condition. Population screening studies suggest that in the UK 1 in 100 people are affected. The complications of untreated coeliac disease can be serious, such as osteoporosis or malignancy.

The treatment of coeliac disease is a lifelong gluten-free 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; access to specialist dietetic support is currently poor within the UK.

1.3. Reasons for the guideline

Currently, people with symptoms and/or signs suggestive of coeliac disease are investigated by serological tests, for example, IgA tissue transglutaminase (tTGA) and IgA endomysial antibodies (EMA), with further referral to a gastrointestinal specialist for endoscopic endoscopic intestinal biopsy to definitively confirm or exclude coeliac disease. There is emerging evidence on the clinical utility of other tests and diagnostic strategies, such as deamidated gliadin peptides (DGP), point of care tests and the use of combined serological tests to definitively diagnose coeliac disease without carrying out endoscopic intestinal biopsy.

The treatment of coeliac disease is a lifelong gluten-free diet. Adherence to a gluten-free diet has repeatedly been shown to be poor, with 20% to 80% of people with coeliac disease admitting to either occasional or prolonged lapses. Specific education and information, such as advice and education on alternative foods in the diet to maintain a healthy and varied intake, which could be provided by a dietitian with experience in coeliac disease, may increase the likelihood of adherence and a positive prognosis.

People with coeliac disease are at risk of complications and may have other coexisting conditions. The follow-up care of people with coeliac disease after the diagnosis varies widely within the UK ranging from follow-up care in specialist clinics to being discharged back to the community without any provision of a follow-up service.

The majority of people with coeliac disease report a rapid clinical improvement after starting a gluten-free diet. However, 5% to 30% do not report symptomatic improvement after starting treatment, and some will still have persisting symptoms after 6 to 12 months. There is currently no conclusive guidance on differential diagnosis of non-responsive coeliac disease, such as infective gastroenteritis, intestinal bacterial overgrowth, lactose intolerance, intestinal lymphoma, and other immunodeficiency conditions. Approximately 10% of people with non-responsive coeliac disease will have true refractory coeliac disease. The management of people with refractory coeliac disease currently varies widely within the UK.

1.4. Population

This guideline covers the following groups:

• Children, young people and adults with symptoms or signs suggestive of coeliac disease.
• Children, young people and adults with confirmed coeliac disease.
• Children, young people and adults considered to be at high risk of coeliac disease. This includes people with autoimmune conditions such as type 1 diabetes and autoimmune thyroid disease, or those with a first-degree family history of coeliac disease.
• Specific subgroups in whom the investigation and management of coeliac disease is known to be different.

The following groups are not covered within this guideline:

• Children, young people and adults with other gastrointestinal disorders (the guideline will only cover differential diagnosis of non-responsive coeliac disease).
• People with non-coeliac disease gluten sensitivity.

For the purposes of the guideline populations have been defined in the following way: children are those below age 16, young people are those aged 16 and 17 years, and adults are those aged 18 years and over.

1.5. Healthcare setting

All settings where NHS healthcare is commissioned or delivered (including a person’s home).

1.6. Medicines

The guideline will assume that prescribers will use a medicine’s summary of product characteristics to inform decisions made with individual patients.

1.7. Patient-centred care

This guideline offers best practice advice on the care of children (below age 16), young people (16 – 17 years of age) and adults (18 years and over) with suspected or confirmed coeliac disease.

Patients and healthcare professionals have rights and responsibilities as set out in the NHS Constitution for England – all NICE guidance is written to reflect these. Treatment and care should take into account individual needs and preferences. Patients should have the opportunity to make informed decisions about their care and treatment, in partnership with their healthcare professionals. If the patient is under 16, their family or carers should also be given information and support to help the child or young person to make decisions about their treatment. If it is clear that the child or young person fully understands the treatment and does not want their family or carers to be involved, they can give their own consent. Healthcare professionals should follow the Department of Health’s advice on consent. If someone does not have capacity to make decisions, healthcare professionals should follow the code of practice that accompanies the Mental Capacity Act and the supplementary code of practice on deprivation of liberty safeguards.

NICE has produced guidance on the components of good patient experience in adult NHS services. All healthcare professionals should follow the recommendations in Patient experience in adult NHS services.

If a young person is moving between paediatric and adult services, care should be planned and managed according to the best practice guidance described in the Department of Health’s Transition: getting it right for young people.

Adult and paediatric healthcare teams should work jointly to provide assessment and services to young people with coeliac disease. Diagnosis and management should be reviewed throughout the transition process, and there should be clarity about who is the lead clinician to ensure continuity of care.