Coeliac Disease

Question 1

Define Coleiac disease

Answer 1

• Chronic, immune-mediated enteropathy thast is triggered and maintained by ingestion of gluten in genetically predisposed individuals found in barley, wheat and rye.

Question 2

What are risk factors for Coeliac disease?

Answer 2

Strong:

• Family members affected
• IgA Deficiency
• Type 1 Diabetes
• Autoimmune Thryoid Disease/Autoimmune diseases in general
• Women (66% of diagnoses)

Others:

• down syndrome
• Turner’s syndrome
• Williams Syndrome
• Osteoporosis
• Iron deficiency Anemia

Question 3

What is meant by silent coeliac disease?

Answer 3

• Disease by where there is villus atrophy of the small intestine however the patient remains aysmptomatic

Question 4

Coeliac is more prevelant in Eurioe and is rare in Africa and Asia. Which part of the british isles is coleiac particulary common?

Answer 4

• Western Ireland

Question 5

What is meant by latent coeliac disease

Answer 5

• Tests show the coeliac disease is likely present, but villi appear normal upon examination during endoscopy.

Question 6

What is gluten (mention amino acids etc)

Answer 6

• Storage protein in plant
• HIgh amino proline content
• Gives dough its visoelastic properties.

Question 7

There are two subclasses of Gluten, name them

Answer 7

Gliadin and Glutenin

Question 8

Try to summarise the key states in pathogenesis coeliac disease - LOOK AT MIND MAP !!!!

Answer 8

MIND MAP

Question 9

State typical presentation of patient with suspected coeliac

Answer 9

• Main one is diarrhoea
  
  • steatorrhoea is common
• abdominal pain
• Bloating
• Indigestion
• Constipation
• Vomiting
• Fatigue, Tiredness
• Weight loss
• Itch
• Difficulty getting pregnant

Question 10

What tests would be performed to test for Coeliac. Important to note that these tests are done when the patient is in a phase of eating gluten, as all diganositc tests will normalise on a gluen free diet.

Answer 10

• Seorlogy Tests
  
  • IgA Tissue Transglutaminas levels should be evlautaed - can also assess for IgA deficiency
  • Endomysial Antbody is another option - more expensive with greater specificity but lower sensitivity
  • In patients with IgA deficiency, request IgG deaminated gliadin peptide levels - this test is not accurate
  • You would also test for HLA-DQ2 and 8 - this seems to be done as a saliva test
• Endoscopy and biopsy
  
  • Macroscopic, changes in small bowel can be seen
  • Microscopic changes - graded based on Marsh Criteria
    
    • Crypt hyperplasia
    • Villus Atrophy
    • Epithelial cells no longer have villi

Question 11

Appreciate

Question 12

The Marsh criteria is used when looking at small bowel under a microscope to examine the histoloigcal status

Question 13

Endoscopy can be avoided in the diagnosis for children with suspected coeliac. When would this be the case?

Answer 13

• Pediatric patients with symptoms consistent with coeliac and a high IgA-tTG tire
  
  • If follow up Endomysial Antibody and Haplotype tests are positive, a diagnosis of coeliac disease can be confirmed without a intestinal biopsy.

Question 14

Are you born with coeliac?

Answer 14

• No, but you are born with genetic pre-disposition
  *

Question 15

The strongest set of test results to support a coeliac diagnosis is:

Answer 15

• Haplotype DQ2 or 8
• IgA tTG positive
• Biopsy positive

Question 16

Potential test combinations. Note that the second combination basically never happens. Obviously with these you would have the accompanying symptoms.

With a tTT positive and biopsy negative then is termed latent coeliac disease

If TtG negative and biopsy positive: Might already be on a gluten free diet, could be other cause of villous atrophy, could also not produce the antibody for tTG, however this extremely rare.

Answer 16

Other causes of villous atrophy

Question 17

So let’s assume coeliac has been diagnosed, what is are the steps now?

Answer 17

• patients referred to dietician - then yearly check-ups to ensure adherence
  
  • Gluten free diet which is considered <20ppm or 20mg/kg
• prescription of oral supplementation to treat any nutritional deficiecnes at diagnosis
  
  • In some people, coeliac makes the spleen work less effectively and are given vaccinations for pneumococcus and meningococcus

Question 18

Coeliac patients are at a higher risk of what degredative bone condition?

Answer 18

Osteporosis

Question 19

Coeliac patients are at a higher risk of infection, potentially due to hypospleenism. To what is the risk of infection higher?

Answer 19

• Pneumococcus
• Meningococcus
• encapsulated organisms
• Haemophillus influenzae

Question 20

What skin rash is associated with coeliac disease ?

Answer 20

Dermatitis Herpetiformis

Question 21

Risk of cancer is increased in coeliac. Which two cancers have been shown to have a clear relation to coeliac?

Answer 21

• Non-hodgkins Lymphoma
• Small bowel Adenocarcinoma

Question 22

8% of People with IgA deficiecny have coeliac. 2-3% of coeliac patients have IgA Deficiency. You should check IgA levels in patient who is tTg negative, and also order IgG subclass autoantibody tests.

Question 23

What is the main reason for coeliac not improving?

Answer 23

Non-compliance with GFD.

See table for others.

Question 24

Stages of investigation for coeliac that is not improving

Question 25

There are cases where the symptoms from coeliac that persist despite adherence to a GFD. Villus atropy remains and can be worse. What is this called ?

Answer 25

Refractory coeliac disease

Question 26

It is also possible that ongoing symptoms are due to cancer of the bowel. What are the two main ones?

Answer 26

• Enteropathy associted lymphoid T cell lymphoma (a complication of coeliac disease in which a malignant T-cell lymphoma develops in areas of the small intestineafflicted by the disease’s intense inflammation)
• Then also tehre is small bowel adenocarcinoma

Question 27

Typical presentation for EATL

Answer 27

Question 28

Prognosis for EATL is poor

Answer 28

Question 29

Treatment for EATL