1. Management of Coeliac Disease in Primary Care Dr Sanjay Gupta Consultant Gastroenterologist

2. Topics covered Epidemiology of coeliac disease Complications and prognosis Management in primary care Prescriptions

3. Coeliac disease March 2016 Scenario: Management of confirmed coeliac disease Age from 6 months onwards Management in primary care

4. How common is it? o The incidence of diagnosed coeliac disease is increasing worldwide. -The prevalence of coeliac disease globally is estimated to be 1% with variations among countries, for example the prevalence of coeliac disease is 2% in Finland and 0.3% in Germany [WGO, 2012]. o Population screening data suggest that the incidence of coeliac disease in the UK is 1 in 100 people [BSPGHAN, 2013]. o Coeliac disease is under-diagnosed, and it is estimated that for every person diagnosed with coeliac disease, seven to eight people remain undiagnosed [Mooney, P. et al., 2014]. o Coeliac disease is approximately twice as common in females than males [Mooney, P. et al., 2014].

6. Prevalence of coeliac

7. What are the complications? Common complications of coeliac disease are caused by malabsorption, such as [NICE, 2015]: o Anaemia, due to deficiency of iron, folate, or vitamin B12. o Osteoporosis, due to malabsorption of calcium and/or vitamin D. Other complications include [Lebwohl, B. et al., 2015; NICE, 2015; SIGN, 2015]: o Increased risk of fragility fractures. o Chronic pancreatitis with pancreatic insufficiency. o Hepatobiliary abnormalities such as autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis. o Splenic dysfunction. o Subfertility. o Bacterial overgrowth. o Lactose intolerance. o Microscopic colitis.

8. o Malignancy, such as Hodgkin's and non- Hodgkin's lymphoma, small intestinal adenocarcinoma, and pancreatic cancer. o Refractory coeliac disease, which is a rare condition, but carries with it an increased risk of lymphoma and high rates of morbidity and mortality. Children may also have [BSPGHAN, 2013]: o Faltering growth. o Delayed puberty.

9. What is the prognosis? o The majority of people with confirmed coeliac disease report a rapid clinical improvement after starting a gluten- free diet, however 5–30% will have ongoing symptoms and some (numbers not specified) will still have persisting symptoms after 6 to 12 months [NICE, 2015]. Life expectancy for people with: o Diagnosed coeliac disease, who are adhering to a gluten- free diet, is the same as for the general population [Mooney, P. et al., 2014]. o Coeliac disease (diagnosed or undiagnosed) who are not adhering to a gluten-free diet, may be less than the general population [Lebwohl, B. et al., 2015]. o Refractory disease — is lower than that of the general population, because of the increased incidence of intestinal lymphoma [WGO, 2012].

10. What are the causes and predisposing factors? Coeliac disease is caused by a heightened immunological response to ingested gluten. The reason for this is not yet known, but predisposing factors have been identified: o Genetic predisposition: There is a close association between coeliac disease and human leukocyte antigens (HLA). Not all people with HLA will develop coeliac disease, however the absence of HLA in a person with suspected coeliac disease means that they do not have, and will never develop, coeliac disease [NICE, 2015]. o People with a first-degree relative with coeliac disease have a 4–17% increased lifetime risk of developing coeliac disease. Of those who are homozygous for HLA DQ2, the estimated risk increases to 26%. The risk is also increased in second-degree relatives, but to a smaller extent and the exact prevalence is not known [Lebwohl, B. et al., 2015].

11. Immunological factors: o People with other autoimmune conditions such as type 1 diabetes mellitus and autoimmune thyroid disease have an increased risk of developing coeliac disease [NICE, 2015].

12. Arrange annual review of a person with confirmed coeliac disease and ensure the following is covered: o Ensure the person has received adequate advice and information on coeliac disease and gluten-free diets. o Assess the person's adherence to a nutritious, varied, gluten- free diet. o Review the type and quantity of gluten-free foods that have been prescribed, and adjust the prescription accordingly. o Do not routinely recommend nutritional supplements to prevent nutritional deficiencies, however: o Advise on high-dose folic acid supplementation (5 mg once daily) for women who are pregnant, or who are planning a pregnancy. o Some people may require specific supplements such as calcium and vitamin D if their dietary intake is insufficient.

13. Assess for symptoms and signs of coeliac disease, its complications and associated conditions: o Ask about gastrointestinal symptoms such as diarrhoea, abdominal pain, and blood in the stools. o Measure body weight, height, and body mass index (BMI) to assess for signs of malnutrition (indicated by weight loss in adults, or faltering growth in children). o Assess and manage osteoporosis risk.

14. Consider annual blood testing, including: o Coeliac serology to help assess adherence to a gluten-free diet. o Full blood count and ferritin — to screen for anaemia. o Thyroid function tests — to screen for autoimmune thyroiditis. o Liver function tests — to screen for autoimmune hepatitis. o Vitamin D, vitamin B12, red cell folate, and serum calcium — to assess for deficiency. o Electrolytes — to assess for Addison's disease.

15. If malabsorption, diarrhoea, or other symptoms recur or persist despite strictly adhering to a gluten-free diet, exclude, diagnose, and/or manage alternative conditions, complications, and specific deficiencies. Refer the person to a gastroenterologist (or paediatric gastroenterologist) if there is: o Faltering growth in a child. o Symptoms and signs suggestive of gastrointestinal cancer such as unexplained blood in the stool — refer under the two-week wait rule. o Complications, or persistent symptoms or signs of coeliac disease despite adherence to a gluten-free diet.

16. Consider referring the person to a dietitian if: o There is difficulty in assessing adherence to a gluten- free diet. o Poor adherence to a gluten-free diet is suspected. Offer immunizations, if indicated, for example for people with splenic dysfunction. For more information see the Public Health England publication of the Green Book: Immunisation against infectious disease. Offer more frequent review if problems arise or if there is a change in clinical needs, for example women who become pregnant.

17. Assessing adherence to a nutritious, varied, gluten-free diet o The NICE GDG recommended, based on clinical experience and expert opinion, that people with coeliac disease and their family/carers should be aware of, and have access to, gluten-free food prescriptions to support adherence to a gluten-free diet. The NICE GDG did not recommend routine nutritional supplements due to limited evidence for their use in addition to a gluten-free diet. It also identified the risk of overdosing on over- the-counter vitamin D, calcium, and iron. o Women with coeliac disease are at an increased risk of neural tube defects due to the malabsorption of folate. It is therefore advised that they use high-dose folic acid supplementation until week 12 of pregnancy

18. Considering annual blood testing The NICE GDG did not find sufficient evidence to change the current practice of annual serology monitoring. It recommended that coeliac serology testing may be used to help assess adherence to a gluten-free diet, but the evidence does not indicate a strong or conclusive relationship between serological titres and dietary adherence. Some people will have persistently raised serological titres despite adhering to a gluten-free diet and vice versa. Managing recurrent or persistent symptoms The NICE GDG found strong evidence for possible underlying causes of non-responsive coeliac disease such as, exposure to gluten, irritable bowel syndrome, microscopic colitis, or refractory coeliac disease.

19. Advise the person with coeliac disease that: o The only effective treatment for coeliac disease is long- term adherence to a gluten-free diet. o It is important to ensure a diet is not only gluten-free but also nutritionally balanced. o A large selection of gluten-free products can be prescribed on the NHS.

20. They must avoid: o Foods based on wheat (and wheat varieties such as spelt, kamut, semolina, triticale), barley, and rye. These include bread, flour, cakes, pastries, and biscuits. o Foods that contain wheat, barley, or rye as fillers or flavouring (such as sausages, ready meals, soups, sauces, and foods cooked with a bread-based batter). o Foods that can be contaminated with gluten during processing and packaging (such as oats) and items fried in the same oil as used to fry gluten-containing food (such as chips from a chip shop that cooks battered fish). o Dietary items that contain malt, such as most beers. Malting is the process of germinating then drying grain such as barley, wheat, and rye. Malting does not remove all of the gluten.

21. o They can include pure, uncontaminated oats in their diet. o Many oats and oat-containing products contain small amounts of gluten from barley, wheat, or rye, and these small amounts can be harmful for people with coeliac disease. o In contrast, pure, uncontaminated oats can be eaten by most people with coeliac disease. o People eating a diet containing of gluten-free oats should be monitored regularly.

22. They should read food labels to check if the products are suitable. o Manufacturers of packaged foods are required by law to list on the product's label any cereal containing gluten that is used as an ingredient, no matter how little is used. o Products using oats as an ingredient must be labelled with 'contains oat gluten', although the protein in oats is avenin. o Food products are suitable for people with coeliac disease if they have the Crossed Grain Symbol, or are labelled 'gluten- free' or 'very low gluten'. o Products containing Codex wheat starch will be labelled as containing wheat or wheat starch, but are suitable for people with coeliac disease. Codex wheat starch is a specially manufactured wheat starch where the gluten in the cereal has been reduced to a trace level.

23. Helpful information and resources are available online, including: o Coeliac UK — a national charity working for people with coeliac disease and dermatitis herpetiformis, which provides support, information, and advice on how best to cope with a gluten-free diet. o Food Standards Agency — provides information about coeliac disease, gluten-free foods, and their labelling.

24. How to prescribe o Usually, following diagnosis, a dietitian will discuss the products available on prescription with the person, and will draw up an initial list of choices. o A list of prescribable gluten-free products can be found on the Coeliac UK website. o Allow the person to try a variety of foods before setting up a repeat prescription. A dietitian or pharmacist may be able to advise on manufacturers who have trial packs of a variety of their products. o Changes may be required to accommodate new needs or preferences. o Gluten-free products are allocated unit values based on their carbohydrate and energy content, and their cost. There is a recommended quantity to prescribe for various patient groups. (Coeliac UK, 2011)

25. Quantity to prescribe o Each prescription should be for a 1-month supply of gluten- free foods. o Gluten-free items are allocated unit values based on their carbohydrate and energy content, and their cost. For example:  400 g bread, rolls, or baguettes — 1 unit.  500 g of flour/cake/bread and pastry mix — 2 units.  100 g biscuits/crackers — 0.5 units.  250 g of pasta — 1 unit.  One 110–180 g pizza base — 0.5 units. o Base the quantity of products on the person's age, gender, activity level, and nutritional needs. o See the tables for children, women and men for examples of minimum monthly prescriptions of gluten-free products.

26. Conclusions NICE guidelines March 2016 have outlined details of annual follow-up in primary care including details of prescriptions Referral to secondary care should be done for patients whose symptoms are not controlled on GF diet No recommendations on who should follow-up

27. Thank you Any questions?