Coeliac disease Flashcards
Define coeliac disease
A systemic autoimmune disease that is triggered by dietary gluten peptides (gliadin part) found in wheat, rye, barley, and related grains
What is the aetiology of coeliac disease
T-cell response to gluten (alcohol-soluble proteins (gliadin) in wheat, barley, rye and oats).
Almost all carry HLA-DQ2 or DQ8
Mostly in the 2nd part of the duodenum. Leads to crypt hyperplasia, loss of villi
What are the risk factors for coeliac disease
Family history of coeliac disease (first degree 6x)
IgA deficiency
T1DM
Autoimmune thyroid disease
Autoimmune liver disease
Down’s syndrome
William’s syndrome
Sjogren’s
IBD
Primary biliary cirrhosis
Dermatitis herpetiformis
What is the epidemiology of coeliac disease
Any age, peaks in childhood and 50-60yrs
1:1 ratio of males to females OR 2/3 females
20% “silent”
1% develop refractory coeliac disease (persists despite a strict gluten free diet)
What are the symptoms of coeliac disease
GI:
- Diarrhoea with foul smelling stools/steatorrhoea
- Abdominal pain
- Bloating
- Nausea and vomiting
- Ulcers on the mouth
Non GI
- Failure to thrive / faltering growth / short stature
- Delayed puberty or menarche
- Weight loss, weakness
- fatigue, irritability
- Dental enamel defects
- Osteoporosis and pathological fractures (vit D)
- itchy rash and dry skin (dermatitis herpetiformis)
What are the signs of coeliac disease on examination
General: osteomalacia, pallor (anaemia)
- Dermatitis herpetiformis (pruritic papulovesicular lesions that occur symmetrically over the extensor surfaces of the arms and legs + buttocks, trunk, neck and scalp)
Height and weight
Face: angular stomatitis, aphthous ulcers
What are the differentials for coeliac disease
Peptic duodenitis
Crohn’s disease
Giardiasis
Small intestinal bacterial overgrowth
Post-gastroenteritis
Tropical sprue
Common variable immune deficiency
Gluten-sensitivity
What investigations should be done for coeliac disease
Bedside: stool sample (negative culture) + faecal calprotectin (elevated)
Bloods:
- IgA (ensure no deficiency)]
- anti-transglutaminase (anti-tTG): raised
- FBC: Fe deficiency anaemia (↓Hb ↓MCV)
- Blood film: microcytic, hypochromic red cells, target cells, Howell-Jolly bodies (hyposplenism)
- endomysial antibody (EMA): raised
- LFTs: ↑AST/ALT
- Vit B12, iron, vit D, folate
- HLA-DQ typing
Other
- Endoscopy and biopsy (DIAGNOSIS): villous atrophy, crypt hyperplasia in SI (flat, smooth appearance)
- Continue eating glucose for at least 6 weeks before
What is the management for coeliac disease
- Gluten free diet (exclude wheat, barley, rye)
- Vitamin supplementation: vit D and calcium
- Immunisation: pneumococcal + booster every 5 years ± influenza
+Follow up - monitor symptoms and repeat serology to check adherence
+ annual blood tests (FBC, ferritin, TFTs, LFTs, B12, folate)
+ support: Coeliac UK
What is a coeliac crisis and what is the management
Usually due to a precipitating major medical event e.g. recent abdominal surgery
Hypovolaemia
Severe watery diarrhoea
Acidosis
Hypocalcaemia
Hypoalbuminaemia
Rehydration + correction of electrolyte abnormalities + corticosteroid e.g. budesonide 9mg orally once daily
Complications of coeliac disease
Anaemia
Impaired growth
Malnutrition, malabsorption
Later menses, amenorrhoea, infertility, and increased risk of miscarriage
Dermatitis herpetiformis
Osteopenia/osteoporosis
Hyposplenism
Increased risk of malignancy e.g. enteropathy-associated T cell lymphoma (EATL)
Neuropathies
Increased risk of renal, eye, and vascular complications in patients with type 1 diabetes and coeliac disease.
What is the prognosis for coeliac disease
Good prognosis
Up to 90% will have complete and lasting resolution of symptoms on a gluten-free diet alone
Ongoing gluten exposure, lactose intolerance and IBS account for 10% of the cases with persistent symptoms
Fewer than 1% of patients can be expected to develop refractory coeliac disease