Leeds Health Pathways

Coeliac Disease - Leeds

1. Background Information / Scope of Pathway

Quick info:
This pathway has been developed by LTHT gastroenterologist Jason Jennings using BSG and NICE Guidelines correct as of 2014.
This pathway has been further updated February 2021. Reviewed and amended by Leeds Nutrition and Dietetics working group with representation from LTHT, LCH, LYPFT. Also discussed and agreed with LTHT Gastroenterologist, Jason Jennings.

2. Information Resources for Patients and Carers

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Gluten Free Diet First Steps Advice - Patient Information Leaflet
Coeliac UK - Web link

3. Development and Updates to this Pathway

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Pathway developed in February 2014
Reviewed February 2015. Reviewed and amended by Leeds Nutrition and Dietetics working group with representation from LTHT, LCH, LYPFT. Also discussed and agreed with LTHT Gastroenterologist, Jason Jennings.

4. Referral Forms, Training and Further Resources

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Referral Forms:
Coeliac Disease Referral Form
Training and Further Resources:
Gluten-Free Foods – Prescribing Guidelines http://nww.lhp.leedsth.nhs.uk/common/guidelines/detail.aspx?ID=6119

5. Suspected Coeliac Disease

Quick info:
Coeliac disease:

is often undiagnosed or misdiagnosed in general practice unless the condition is actively considered
should also be considered before a diagnosis of irritable bowel syndrome (IBS) is made

Clinical presentation:

many patients have minimal symptoms or present atypically
occurs frequently without gastrointestinal (GI) symptoms

in adults, coeliac disease may be diagnosed on average over 10 years after first symptoms

Adult disease may present with chronic or unexplained gastrointestinal symptoms, or various associated complications, including:

anaemia:

at least 50% of patients have anaemia at presentation
iron deficiency the commonest picture followed by low folate, which may result in a mixed population
B12 deficiency is rare as absorption is co-factor dependent and occurs in the often unaffected terminal ileum
haemoglobin levels return to normal with a gluten free diet

"IBS type symptoms"
isolated nutritional deficiency, eg:

hypocalcaemia
folic acid deficiency
vitamin D deficiency

dermatitis herpetiformis:

itchy, blistering rash on trunk and extensor surfaces
cutaneous manifestation

autoimmune diseases, eg type 1 diabetes mellitus, thyroid disease
unexplained osteoporosis and fragility fractures
lactose intolerance, pancreatic insufficiency, microscopic colitis
reduced fertility, adverse pregnancy outcomes, recurrent abortion, or amenorrhoea
unexplained elevated transaminases, indicating either a non-specific hepatitis or associated unrecognised autoimmune hepatitis
unexplained neurological or psychiatric symptoms
sexual and reproductive dysfunction

6. When to Offer Serological Testing

Quick info:
Population screening for coeliac disease is not currently recommended.
Suspect coeliac disease, and offer serologic testing, if the person has any of the characteristic symptoms and signs:

sudden or unexpected weight loss - being overweight does not rule out the possibility of coeliac disease
failure to thrive or faltering growth (in children)
chronic or intermittent diarrhoea
recurrent abdominal pain, cramping, or distension
gastrointestinal symptoms, including nausea and vomiting, that are persistent or unexplained
prolonged fatigue (tired all the time)
unexplained anaemia (iron, vitamin B12, or folate deficiency)

Coeliac disease presents more often with atypical symptoms; therefore, test people who are known to be at increased risk because they have:

dermatitis herpetiformis
autoimmune thyroid disease
symptoms of irritable bowel syndrome
type 1 diabetes
a first-degree relative (parent, sibling, or child) with coeliac disease

Consider testing for coeliac disease if the person has any of the following conditions which are known to be associated with coeliac disease:

autoimmune conditions:

Addison's disease
autoimmune liver conditions
autoimmune myocarditis
chronic thrombocytopenia purpura
Sjögren's syndrome

problems that could be due to an underlying autoimmune condition:

persistently elevated liver enzymes with unknown cause.
polyneuropathy
unexplained alopecia
unexplained subfertility

gynaecological conditions:

amenorrhoea
recurrent miscarriage

bone conditions:

low-trauma fracture
reduced bone mineral density (especially in men and premenopausal women)
metabolic bone disease (such as rickets or osteomalacia)

gastrointestinal conditions:

aphthous stomatitis (mouth ulcers)
dental enamel defects
microscopic colitis
persistent or unexplained constipation

genetic conditions:

Down's syndrome
Turner syndrome

miscellaneous conditions:

depression or bipolar disorder
epilepsy
lymphoma
sarcoidosis

7. History and examination

Quick info:
Assessment of adults with suspected coeliac disease:

ask patient about:

frequency and duration of symptoms, including:

chronic or intermittent diarrhoea
recurrent abdominal pain, cramping or distension
weight loss
chronic GI symptoms including nausea and vomiting
chronic fatigue, low mood, poor appetite
steatorrhoea

whether they have noted any dietary precipitants for symptoms
associated features, including:

iron or folate deficiency anaemia
family history of coeliac disease
aphthous ulcers
abnormal levels of dental decay
type 1 diabetes
autoimmune thyroid disease
infertility
neurological disorders
osteoporosis and osteomalacia
abnormal clotting secondary to malabsorption
dermatitis herpetiformis
irritable bowel syndrome

check body weight, height and body mass index (BMI) to assess for signs of malnutrition
examine for:

angular stomatitis
skin rashes, including dermatitis herpetiformis
evidence of anaemia
abdominal tenderness and distension

8. Serological testing

Quick info:
Before testing for coeliac disease:

confirm that the person has eaten gluten-containing foods (with wheat, barley, or rye as an ingredient) e.g. at least 4 slices of bread a day for a minimum of 4 weeks before testing
ensure that the person understands what coeliac disease is
If on a gluten free diet there may be a false negative result
explain that:

serological tests (including commercially available self-tests) do not diagnose coeliac disease, but help indicate whether further testing is needed
if the test is positive, they will be referred for upper GI endoscopy
if the test is negative, coeliac disease is unlikely, but it could develop in the future

NB: Seronegative coeliac disease does exist.
Send blood for coeliac disease serology:

the National Institute for Health and Clinical Excellence (NICE) recommends immunoglobulin A tissue transglutaminase antibody (IgA tTGA) as the first choice test [3]:

NICE advises reserving immunoglobulin A endomysial antibodies (IgA EMA) testing if the result of the IgA tTGA test is equivocal

Leeds pathology will test IgA EMA automatically if IgA tTG is equivocal

IgA deficiency leads to false negative results from IgA tTGA or IgA EMA tests:
If strong clinical suspicion but negative serology consider checking IgA

9. Positive result - Advise Patient Not to Start Gluten Free Diet

Quick info:
Following a positive serology test:

offer referral to gastrointestinal specialist for endoscopy and biopsy of small intestine to confirm or exclude coeliac disease
advise the patient not to start a gluten-free diet until diagnosis is confirmed by intestinal biopsy

10. Negative Result Seek Alternative Diagnosis

Quick info:
Note:

testing for coeliac auto-antibodies is satisfactory to exclude coeliac disease in the majority of cases as the best serological tests have 95% negative predictive value
if serology tests are negative, advise that this excludes coeliac disease at the moment, but it does not rule out the development of coeliac disease in the future
seronegative coeliac disease does exist
if there is a high clinical suspicion of coeliac disease despite repeatedly negative serology, formal referral to specialist gastroenterologist for assessment and duodenal biopsy is advised

11. High Clinical Suspicion despite Negative / Equivocal Serology

Quick info:
Options include:

Refer for gastroenterologist opinion
Offer Gluten Challenge (4 slices of bread per day for minimum of 4 weeks) and repeat blood test
Repeat test after set interval (minimum of 6 months.)

12. Investigations to Accompany Referral

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Please consider the following immediate investigations to accompany the referral:

full blood count (FBC)
urea and electrolytes
liver function tests (LFTs)
serum calcium and vitamin D
clotting studies
haematinics
thyroid function tests (TFTs)
Upper GI endoscopy if patient is fit and willing and on a normal diet

13. Refer To Gastroenterology

Quick info:
Coeliac Disease Referral Form
Patients should not be routinely referred to direct access diagnostic providers for upper gi endoscopic procedure to confirm a diagnosis of coeliac disease.
In the event that direct access diagnostic providers, suspect coeliac disease following an endoscopic procedure, they should liaise with primary care to ensure accompanying blood test results and DEXA Scan to aid diagnosis by gastroenterology services and /or dietetic services. Community diagnostic providers are not responsible for initial patient management at this point but are responsible for onward referral recommendation.

14. Initial Patient Management

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First Clinic appointment may include the following:
Establish diagnosis
History
Review investigations or take bloods if not taken previously - consider tTG, HLA, D2 biopsy
If anaemic consider referal for colonoscopy.
Full Blood Count
B12 Folate Ferritin
U&E
LFT
Ca PO4 Vit D
TFT
tTG
DXA
Dietetic review - commence gluten free diet.
Consider Calceos T bd po
Treat deficiencies Coeliac UK membership Coeliac UK buddy scheme etc
Recommend GFD prescriptions
Recommend PneumoVax
Recommend screen first degree relatives.
Discuss Gluten Free prescription options
http://nww.lhp.leedsth.nhs.uk/common/guidelines/detail.aspx?ID=6119

15. Subsequent Follow-up Management

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Subsequent hospital clinic appointment may include the following:
Review of progress
Review gluten free diet
Review problems
Further dietetic follow-up on case by case basis
Consider repeat endoscopy at 18 to 24 months on gluten free diet.

16. Who to Discharge From Routine Follow-up

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Who to discharge?
Where there is a clear diagnosis (specialist may decide to keep latent/silent coeliac patients under review) Symptom resolved
Well motivated with diet.
Good histological response - if second endoscopy.
Dietetic Review
LTHT gastroenterologists suggest current best practice advice is to review coeliac patients at one year post discharge via a dietetic review – patients may therefore be discharged from routine follow-up once stable for a dietetic review one year on (see section 18).

17. Symptoms Persist - Continued Gastroenterology Management

Quick info:
Points to consider:

Is it really coeliac disease - consider other causes of villous atrophy
Dietetic review
Other causes of diarrhoea associated with coeliac disease (e.g. microscopic colitis, pancreatic insufficiency, lactose intolerance)
Refractory coeliac disease
Complications of coeliac disease (e.g. ulcerative jejunitis, lymphoma)
Consider advice and guidance request or referral into secondary care gastroenterology services
Bone density scanning is recommended for women at the menopause and males at aged 55 with coeliac disease. If a scan is abnormal a further scan should be arranged every three years.

Appropriate actions may include:

arranging blood tests
correcting any deficiencies e.g. Ca, Vit D, folic acid etc
consider IBS medication e.g. colpermin, buscopan, amitryptiline if bloating
arranging a bone density scan if appropriate
arranging pneumococcal vaccination as necessary
If clinical concern - refer to gastroenterology. See next box for more details including red flag signs

18. Dietetic Review Post Discharge from Routine Follow-up (Patient Stable)

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Annual Dietetic Review
LTHT gastroenteriologists suggest current best practice advice is to review coeliac patients one year post discharge as stable.
Annual Dietetic Review

19. Criteria For Re-Referral To Gastroenterology

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Return to clinic if:

Ongoing symptoms after dietetic review.
New symptoms after dietetic review.
Red flag symptoms - Clinicians should be aware of the possibility of developing associated autoimmune disease and small bowel lymphoma and investigate and refer patients with further symptoms accordingly. 2ww colorectal cancer pathway may also be considered.
Recurrent anaemia.

Key Dates
Published: 17-Feb-2015, by Leeds
Valid until: 01-Jun-2016
Reviewed and republished – March 2021