The Management of Adults with Coeliac Disease in Primary Care

Primary Care Society for Gastroenterology

The purpose of this document is to assist healthcare professionals who are responsible for the diagnosis and management of patients with coeliac disease. The document supersedes PCSG guidelines on:

Decision points in the management of adult coeliac disease in primary care
Follow-up care of adult coeliac disease

Introduction

Coeliac disease is a chronic, permanent and if untreated, potentially life-threatening condition. In coeliac disease, the mucosa of the small intestine is damaged by gluten, a protein found in wheat, rye and barley. A similar protein in oats may cause damage in people with severe sensitivity to gluten.

The damage to the small intestine results in the reduced ability to digest and absorb food and causes malabsorption of essential nutrients such as vitamins, iron, folic acid and calcium.

Key facts about coeliac disease

Coeliac disease affects as many as one in 100 people in the UK^1,2 [C]
The only treatment for coeliac disease is a strict, life-long gluten-free diet. Effective management therefore relies on regular follow-up of patients to ensure strict adherence^3,4,5 [D]
Untreated coeliac disease often results in unnecessary morbidity⁶ [B] including:
- Faltering growth in childhood⁷ [C]
- Osteopenia⁸ [C]
- Osteoporosis^9,10,11 [C]
- Development of autoimmune disorders^12,13 [C]
- Malignancy^14,15 [C]
- Anaemia^6,16 [B]

Symptoms

Coeliac disease is often undiagnosed or misdiagnosed in general practice unless the condition is actively considered. Coeliac disease should also be considered and if necessary discounted before a diagnosis of Irritable Bowel Syndrome is made⁶.

Coeliac disease should be considered in patients presenting with:

Iron or folate deficiency anaemia
Tired all the time (‘TATT') or chronic fatigue
Unexplained diarrhoea
Abnormal levels of dental decay
Persistent mouth ulcers

And particularly if the patient also has:

Family history of coeliac disease
Type 1 diabetes
Autoimmune thyroid disease
Osteoporosis
Infertility
Unexplained neurological disease

Diagnosis

Initial diagnosis is by means of a simple blood test for gliadin antibodies. Endomysium antibody (EMA) and tissue transglutaminase (TTG) are non-invasive tests that provide a simple means of screening for coeliac disease¹⁷ [C]. The EMA test is the initial screening test of choice. It may, however, be negative in 2% of people with coeliac disease who are IgA deficient. Therefore consider requesting an IgA level at the same time. TTG is also useful as a preliminary screening step for coeliac disease
For patients with an uncertain diagnosis, test by EMA after six weeks on a diet which includes four slices of normal bread per day for a minimum of two weeks. If the result is positive then intestinal biopsy should be offered³ [D]
Following a positive EMA test, a definitive diagnosis can be made by patient referral to a gastroenterologist for a small intestinal biopsy

Management

Once diagnosis is confirmed, coeliac disease is managed by following a strict gluten-free diet which should resolve many of the symptoms and improve the patient's health outcome.

Benefits of long-term adherence to a gluten-free diet

Effective management of coeliac disease relies on regular follow-up of patients to ensure strict adherence to a gluten-free diet^3,4,5 [D]. Other reasons to ensure adherence include:

The risk of developing the long-term complications associated with coeliac disease, as previously mentioned, is likely to be reduced and the individual is likely to return to full health^8,10,15,18 [C]
Almost 50% of all coeliac patients have an inadequate energy intake, and 11% have an inadequate intake of calcium and vitamin B6. 80% of patients with coeliac disease have an inadequate intake of vitamin D¹⁹ [C]
Regular follow-up visits to monitor adherence by a health professional has a positive impact and helps the patient maintain a gluten-free diet^20,21,22,23 [C]

Clinical monitoring of adherence to a gluten-free diet

Serological tests to measure endomysial and tissue transglutaminase antibodies are an accepted and reliable marker for dietary adherence^17,24 [C]
A small intestinal biopsy should be performed four to six months after initiating a gluten-free diet. Improvement in the villous architecture of the small intestine would be expected

Follow-up

Patient adherence with a gluten-free diet is poor; ranging from 45-87%^20,21 [C].

Good dietary adherence is aided by the ease with which patients can obtain appropriate amounts of gluten-free products on prescription²⁵ [D]. Patients should therefore be followed up throughout their lifetime so that the primary healthcare team can ensure long-term adherence to a gluten-free diet.

Regular follow-up is also an opportunity to provide patient-centred care that is sensitive to the individual's circumstances.

Frequency

After diagnosis, the patient should be reviewed at the hospital gastoenterology clinic after three months and six months to ensure that they are making satisfactory progress and managing the diet
Pregnancy is a particularly important time for patients to be reviewed
If the patient is well, they should then be reviewed annually in primary care or sooner if problems arise

Organisation

Create a database of coeliac disease patients to facilitate recall and audit
Use a template to record clinical data in a standardised way, in order to facilitate audit and research
Have a named person who will have clinical and administrative responsibility for the service
The service should be audited annually: audit standards may include a number of patients who have recorded evidence of:
- Compliance with a gluten-free diet
- Nutritional status
- Body mass index
- Osteoporosis assessment

Annual assessment tests

The annual assessment should involve assessment of, and motivation towards, strict adherence to a gluten-free diet. The prescription should be reviewed to ensure it is appropriate (see Table 1)²⁶. Development of long-term complications should be monitored.

Coeliac disease status

Measure weight, height and body mass index⁵ [D]
Assess symptoms; bowel function (stool frequency, consistency, blood in stool)⁵ [D]
Conduct investigations: haemoglobin, red cell folate, serum ferritin, serum albumin and alkaline phosphatase.
Patients with coeliac disease who adhere to a gluten-free diet often consume inadequate intakes of folic acid and iron¹⁹ [B]
Assess gliadin, endomysial or tissue transglutaminase antibodies to monitor significant dietary gluten ingestion¹⁷ [C]
Conduct routine blood tests into possible nutritional deficiencies ie calcium, vitamin D and B12³ [D]

Disease prevention: osteoporosis risk assessment and management

Bone mineral density should be measured using dual x-ray absorptiometry (DEXA) at the time of diagnosis. The test should then be repeated:
- At the menopause for women
- At the age of 55 years for men
- At any age should a fragility fracture occur
Advise regular physical activity, reduce smoking and alcohol consumption²⁷ [B]
Include a calcium supplement if required^27,28 [A]
Vitamin D supplements can be prescribed if the patient is housebound [D]
Hormone replacement therapy and biphosphonates should be considered if the patient is osteoporotic [D]

Because there is some degree of splenic atrophy in most patients with coeliac disease, it can be sufficiently severe to cause peripheral blood changes in about 25%. Patients should therefore be considered for:

Vaccination against pneumococcus and haemophilus influenzae type b [D]
Vaccination against influenza [D]
Guidance about the increased risks attached to tropical infections eg malaria [D]
Life-long prophylactic antibiotics are not recommended [D]
Management of associated medical problems
Discussion of familial risk if required. First-degree relatives of people with coeliac disease have a 1 in 10 chance of developing the disease and should be assessed if they develop suggestive symptoms

The role of the primary healthcare team

GP

The GP can ensure that:

At least the recommended minimum monthly amount of gluten-free products is prescribed to enable the patient to adhere to a balanced and healthy gluten-free diet (see Table 1)²⁶ [D]. Patients have varying requirements for gluten-free foods depending on their age, gender, occupation and lifestyle
The patient is reviewed annually with or without the input of the gastroenterologist and/or dietitian, depending on the needs of the patient

Practice nurse

The practice nurse can:

Provide good, sensible, practical advice and refer to the dietitian and/or GP as required
Assist and support patients in the adherence to a strict gluten-free diet
Provide a first point of contact for the patient between medical and dietetic appointments
Monitor the prescription of gluten-free foods and anthropometry (weight, body mass index)
Arrange blood tests

Dietitian

The dietitian has an important role in follow-up care by:

Ensuring a balanced, varied gluten-free diet
Reviewing the dietary needs regularly and recommending changes to the prescription if required (this will require input from the GP)²⁶
Assessing the nutritional status
Monitoring the weight and body mass index
Referring to the GP if problems arise
Discussing Coeliac UK membership
Advising on the use of Coeliac UK's Food and Drink Directory
Providing general advice on food labelling

Pharmacist

The pharmacist can:

Input to the prescription needs of the patient²⁶ [D]
Liaise with the healthcare team managing the patient
Provide general advice and support to the patient

Referral to the gastroenterologist

Referral should be considered if there is:

Poor response to the gluten-free diet
Weight loss on a gluten-free diet
Blood in the stools
Onset of unexplained abdominal pain
Abnormalities in blood results
Other clinical concerns

Useful sources of information

Gluten-free foods: a prescribing guide. February 2004 (available via local PCT and PCSG)
Coeliac UK. www.coeliac.org.uk
Primary Care Society for Gastroenterology. www.pcsg.org.uk

British Society for Gastroenterology. www.bsg.org.uk

People with coeliac disease: minimum monthly gluten-free food prescription requirements
Age group	Suggested no. units per month*	Example minimum monthly prescription (items can be interchanged e.g. bread mixes can be interchanged with rolls or loaves
Child 1-3 Years	10	4 x 400g loaves of bread (or 2 x 500g mix suitable for making bread) 1 x 500g pasta 2 x 200g biscuits 1 x 500g flour mix
Child 4-6 Years	11	4 x 400g loaves of bread (or 2 x 500g mix suitable for making bread) 2 x (2 x 110/180g) pizza bases 3 x 200g biscuits 1 x 500g flour mix
Child 7-10 Years	13	6 x 400g loaves of bread (or 3 x 500g mix suitable for making bread) 1 x 500g pasta 1 x (2 x 110/180g) pizza bases 2 x 200g biscuits 1 x 500g flour mix
Child 11-14 Years	15	6 x 400g loaves of bread (or 3 x 500g mix suitable for making bread) 2 x (2 x 110/180g) pizza bases 3 x 200g biscuits 1 x 500g flour mix 1 x 500g pasta
Child 15-18 Years	18	6 x 400g loaves of bread (or 3 x 500g mix suitable for making bread) 2 x (2 x 110/180g) pizza bases 4 x 200g biscuits 2 x 500g flour mix 1 x 500g pasta
Male 19-59 Years	18	10 x 400g loaves of bread (or 5 x 500g mix suitable for making bread) 1 x (2 x 110/180g) pizza bases 1 x 500g pasta 2 x 200g crackers/crispbreads 1 x 200g sweet biscuits 1 x 500g flour mix
Male 60-74 Years	16	10 x 400g loaves of bread (or 5 x 500g mix suitable for making bread) 1 x 500g cake mix 1 x 500g pasta 1 x 200g crackers/crispbreads 1 x 200g sweet biscuits
Male 75+ Years	14	8 x 400g loaves of bread (or 4 x 500g mix suitable for making bread) 1 x 500g cake mix 1 x 500g pasta 1 x 200g crackers/crispbreads 1 x 200g sweet biscuits
Female 19-74 Years	14	8 x 400g loaves of bread (or 4 x 500g mix suitable for making bread) 1 x (2 x 110/180g) pizza bases 1 x 500g pasta 1 x 200g crackers/crispbreads 1 x 200g sweet biscuits
Female 75+ Years	12	6 x 400g loaves of bread (or 3 x 500g mix suitable for making bread) 1 x 500g cake mix 1 x 500g pasta 1 x 200g crackers/crispbreads 1 x 200g sweet biscuits
Breastfeeding	Add 4 units	1 x 500g pasta 1 x 200g crackers/crispbreads 1 x 400g loaf of bread
3rd Trimester Pregnancy	Add 1 unit	1 x 200g sweet biscuits
High physical activity level	Add 4 units	1 x 500g pasta 1 x 200g sweet biscuits 1 x 200g crackers/crispbreads

References

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3. Guidelines for the management of patients with coeliac disease. British Society of Gastroenterology. 1998, 2002 www.bsg.org.uk
4. Decision points in the management of adult coeliac disease in primary care. Primary Care Society of Gastroenterology. 1999 www.pcsg.org.uk
5. Follow-up care of adult coeliac disease. Primary Care Society for Gastroenterology. 2001 www.pcsg.org.uk
6. Hin H, Bird G, Fisher P et al. Coeliac disease in primary care: case finding study. British Medical Journal 1999; 318: 164-167
7. Walker-Smith JA, Guandalini S, Schmitz J et al. Revised criteria for diagnosis of coeliac disease. Archives of Disease in Childhood 1990; 65(8): 909-911
8. Valdimarsson T, Lofman O, Toss G et al. Reversal of osteopenia with diet in adult coeliac disease. Gut 1996; 38: 322-327
9. Valdimarsson T, Toss G, Ross I et al. Bone mineral density in coeliac disease. Scandanavian Journal of Gastroenterology 1994; 29: 457-461
10. McFarlane XA, Bhalla AK, Reeves DE et al. Osteoporosis in treated adult coeliac disease. Gut 1995; 36: 710 -714
11. Stenson WF, Newberry R, Lorenz R et al. Increased prevalence of coeliac disease and need for routine screening among patients with osteoporosis. Arch Intern Med 2005; 165(4): 393-399
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13. Cuoco L, Cero M, Jorizzo et al. Prevalence and early diagnosis of coeliac disease in autoimmune thyroid disorders. Italian Journal of Gastroenterology and Hepatology 1999; 31: 283-287
14. Catassi C, Bearzi I and Holmes G. Gastroenterology. 2005; 128: S79-86
15. Holmes GKT, Prior P, Lane MR, et al. Malignancy in coeliac disease - the effect of a gluten-free diet. Gut 1989; 30: 333-338
16. Maki M, Collin P. Coeliac disease. Lancet 1997; 349: 1755-1759
17. Collin P, Kaukinen K, Vogelsang H et al. Antiendomysial and antihuman recombinant tissue transglutaminase antibodies in the diagnosis of celiac disease: a biopsy proven European multicentre study. European Journal of Gastroenterology and Hepatology 2005; 17(1): 41-43
18. Sheldon W. Prognosis in early adult life of coeliac disease with a gluten-free diet. British Medical Journal 1969; i: 401-404
19. Xola, A et al. Journal of Human Nutrition and Dietetics 1995; 8: 231-237
20. Bardella MT, Molteni N, Prampolini L et al. Need for follow-up in celiac disease. Arch Dis Child 1994; 70: 211-213
21. Ljungman G, Myrdal U. Compliance in teenagers with coeliac disease - a Swedish follow-up study. Acta Paediatr 1993; 82: 235-238
22. Case S. The gluten-free diet:how to provide effective education and resources. Gastroenterology 2005; 128: S128-134
23. Pietzak MM. Follow-up of patients with coeliac disease: achieving compliance with treatment. Gastroenterology 2005; 128: S135-141
24. Baudon JJ, Johanet C, Absalon YB et al. Diagnosing celiac disease: a comparison of human tissue transglutaminase antibodies with antigliadin and antiendomysium antibodies. Archives of Paediatric Adolescent Medicine 2004; 158(6): 584-588
25. The role of gluten-free foods in coeliac disease: the evidence. May 2002. NDC 151
26. Gluten-free foods: a prescribing guide. February 2004
27. Scott EM and Scott BB. A strategy for osteoporosis in gastroenterology. European Journal of Gastroenterology and Hepatology 1998; 10(8): 689-698
28. McFarlane,XA., Bhalla AK, Robertson DAF. Gut 1996; 39: 180-184

The strength of these recommendations is based on the quality of supporting evidence:

A evidence from randomised controlled trials
B evidence from other controlled or quasi-experimental studies
C evidence from descriptive studies
D expert opinion and clinical experience

Primary Care Society for Gastroenterology www.pcsg.org.uk
Date of publication: May 2006