Coeliac Disease

Question 1

Definition

Answer 1

Systemic autoimmune disease triggered by dietary gluten peptides & related grains. Locally causing GI upset & symptoms of malabsorption and a systemically diverse range of symptoms that can affect every organ system.

Question 2

Prevalence & demographics

Answer 2

Uncommon
M=F but more females affected
2peaks: infants, 40-50yrs

Question 3

Cause

Answer 3

Gluten Peptides
Genetics (HLA-DQ2, DQ8)
?GI infection
?Direct damage to intestinal epithelial barrier

Question 4

Pathophysiology

Answer 4

• Loss of immune tolerance to gliadin peptide antigens
• from wheat/barley.
• Peptides resistant to proteases
• stay intact in the s.bowel lumen.
• In intestinal submucosa peptides trigger innate & adaptive immune activation
• leads to epithelial damage.
• Gliadin is de-amidated binding to coeliac associated HLA peptides (DQ2, DQ8)

Question 5

Risk factors

Answer 5

FHx
IgA deficiency
Type1 diabetes
Autoimmune thyroid disease
Down’s syndrome
Sjorgren’s syndrome
IBD
Primary biliary cirrhosis

Question 6

Clinical presentation

Answer 6

Bloating, abdo pain/discomfort

Anaemia, osteopenia/osteoporosis, weight loss, fatigue, failure to thrive, diarrhoea

Question 7

Signs

Answer 7

IgA deficiency, ataxia, alopecia, peripheral neuropathy (uncommon)

Question 8

Differentials

Answer 8

Peptic duodenitis
Crohn’s
Giardiasis
S. GI bacterial overgrowth
Eosinophilic enteritis
Post-GE
Tropical sprue
CVID & immunodeficiency

Question 9

Investigations

Answer 9

Bloods: FBC (low Hb, microcytic RBC), IgA-tTG (titre above normal), EMA (elevated), IgG-tTG (elevated), IgG DGP (elevated)
Skin biopsy: Granular deposits of IgA at dermal papillae of lesional skin
Consider: HLA typing, S.bowel histology & macroscopic

Question 10

Management

Answer 10

Ongoing: Gluten free diet, Ca, iron & vitD supplements
Refractory: ABOVE, nutritionist & gastroenterologist input
Coeliac Crisis: ABOVE, rehydration, correct electrolyte imbalances (acidosis, hypocalcaemia & hypoalbuminaemia), corticosteroids (Prednisolone, budesonide)

Question 11

Prognosis

Answer 11

Good- manageable with gluten free diet alone 90% of the time.

Question 12

Complications

Answer 12

Malignancy
Osteoporosis/ osteopenia
Dermatitis herpetiformis
Idiopathic recurrent acute/chronic pancreatitis