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Coeliac Disease Coeliac Disease (Gluten Sensitive Enteropathy) Coeliac Disease (CD) is an immunologically mediated disorder in which there is inflammation of mucosa of the upper small bowel (duodenum and jejunum) that improves when gluten is withdrawn from the diet and relapses when gluten is reintroduced Up to 1% of population is affected
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Coeliac Disease (CD)By Dr. Zahoor
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Coeliac DiseaseCoeliac Disease (Gluten Sensitive
Enteropathy)•Coeliac Disease (CD) is an immunologically
mediated disorder in which there is inflammation of mucosa of the upper small bowel (duodenum and jejunum) that improves when gluten is withdrawn from the diet and relapses when gluten is reintroduced
•Up to 1% of population is affected
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Coeliac Disease (Gluten Sensitive Enteropathy)
What is Gluten? Gluten is the protein content of the
cereals, present in Wheat, Barley, Rye and Oat
Prolamins (seed protein) – damaging factors
Gliadin – from wheat Hordeins – from Barley Secalins – from Rye
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BarleyRye
Oats Wheat
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Coeliac Disease (Gluten Sensitive Enteropathy)
•Gluten protein present in wheat, barley, rye can not be digested by pepsin, chymotrypsin because of their high glutamine and proline contents, therefore, remain in the intestine lumen triggering immune response
Immunology•Gliadin peptides pass through epithelium in
small intestine and bind to antigen presenting cells
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Coeliac Disease (Gluten Sensitive Enteropathy)
•Antigens present in cells interact with CD4 T-cell ( T- helper cell) in the lamina propria
•These CD4 T-cell produce interferon and also interact with B-cell to produce antibodies
•Gliadin peptide also causes release of interleukin – 15 from entrocytes, activating entroepethilial lymphocytes
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Coeliac Disease (Gluten Sensitive Enteropathy)•This inflammatory cascade releases
metalloproteinases and other mediators that contribute to the VILLIOUS ATROPHY and CRYPT HYPERPLASIA, which are typical of Coeliac disease.
•Mucosa of proximal small bowel (duodenum and jejunum) is predominantly affected. Mucosal damage decreases towards ileum.
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Pathophysiology of Coeliac Disease
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Coeliac Disease (Gluten Sensitive Enteropathy)
Genetic Factors•Increase incidence of Coeliac Disease
occurs in families but exact mode of inheritance is not known
•Over 90% of patients will have HLA-DQ2
Environmental Factors•Rota virus infection in infancy increases
the risk
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Coeliac Disease (Gluten Sensitive Enteropathy)Clinical Features•Coeliac Disease can present at any age •In infancy, it sometimes appears when
gluten containing foods are introduced •In adult, peak age of diagnosis is 5th decade
with F > M. Many patients are asymptomatic (silent) and come to attention because of routine blood test e.g. raised MCV, or iron deficiency in pregnancy
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Coeliac Disease (Gluten Sensitive Enteropathy)
Symptoms•These are non-specific e.g. tiredness and
malaise associated with anaemia
GI Symptoms •May be absent or mild•Diarrhoea, Steatorrhea, abdominal pain and
weight loss – suggest more severe disease•Mouth ulcer, angular stomatitis are frequent
and can be intermittent
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Coeliac Disease (Gluten Sensitive Enteropathy)• Neuropsychiatric symptoms of anxiety and
depression occur
Neurological symptoms• Paraesthesia, Ataxia, Muscle weakness,
polyneuropathy occur
• Other Symptoms – Infertility
Rare complications• Tetany• Osteomalacia, Osteoporosis• Gross mal nutrition with peripheral edema
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Coeliac Disease (Gluten Sensitive Enteropathy)
Association with other autoimmune disease•There is increased incidence of autoimmune
diseases associated with CD, like - Thyroid disease - Type I Diabetes Mellitus - Sjogren’s syndrome
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Coeliac Disease (Gluten Sensitive Enteropathy)
Other Associated Diseases with CD•Inflammatory bowel disease •Primary Biliary cirrhosis •Chronic liver disease•Interstitial lung disease •Epilepsy
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Coeliac Disease•Consider Differential Diagnosis - Giardiasis - Chronic pancreatitis - Lactose intolerance - Irritable bowel syndrome - Crohn disease
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Coeliac Disease (Gluten Sensitive Enteropathy)
Diagnosis•Small bowel biopsy Histology •Shows subtotal villous atrophy•Crypt hyperplasia with inflammatory
cells, increased lymphocyte and plasma cells in the lamina propria
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Coeliac Disease (Gluten Sensitive Enteropathy)
Diagram showing normal and subtotal villous atrophy
Normal Mucosa Subtotal Villous Atrophy in CD
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Coeliac Disease (Gluten Sensitive Enteropathy)
Causes of Subtotal Villous Atrophy
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Coeliac Disease (Gluten Sensitive Enteropathy)
Diagnosis (cont)Endoscopy •Endoscopy shows absence of mucosal folds,
mosaic pattern of mucosal surface
Serology•Endomysial and anti tissue transglutaminase
antibodies – sensitivity is more than 90% •IgA antibodies
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Coeliac Disease (Gluten Sensitive Enteropathy)
DiagnosisHLA typing•HLA - DQ2 ( gene marker) is present in 90 - 95%
cases ( on chromosome-6 )•HLA – DQ8 is present in about 8%
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Coeliac Disease (Gluten Sensitive Enteropathy)
Other investigation•Folate deficiency is common and causes
macrocytosis (Vitamin B12 deficiency is rare)•Iron deficiency is common•Blood film may show microcytosis and
macrocytosis, hyper segmented polymorphonueclear leukocytes
•Howell – Jolly bodies due to splenic atrophy
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Howell – Jolly bodies in a blood smear stained with Giemsa
Note: Howell-Jolly bodies are histological finding. It is basophilic nuclear remanants (cluster of DNA) in circulating erythrocytes.
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Coeliac Disease (Gluten Sensitive Enteropathy)
Investigations (cont)
Biochemistry•Low calcium and high phosphate –
Osteomalacia•Hypoalbuminaemia
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Coeliac Disease (Gluten Sensitive Enteropathy)
Investigation (cont)
Radiology• Small bowel follow through may show
dilatation of small bowel with slow transit •Bone densitometry (DXA) – should be
performed due to risk of Osteoporosis
•Capsule endoscopy
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Coeliac Disease (Gluten Sensitive Enteropathy)
Treatment and Management• Gluten free diet for life Elimination of wheat, barley, rye ,oatsNOTE – Diet produces clinical improvement within
days or weeks. Morphological improvement takes months
• Replacement of minerals and vitamins E.g. Iron, folic acid, calcium, vitamin D
• Pneumococcal vaccination – once every 5 years (because of splenic atrophy)
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Coeliac Disease (Gluten Sensitive Enteropathy)
Complications•Very rarely, T-cell lymphoma •Ulcerative jejunitis – present with fever,
abdominal pain, perforation and bleeding
Diagnosis for these Complications•MRI •Laparoscopy with small bowel biopsy •Barium studies
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Coeliac Disease (Gluten Sensitive Enteropathy)
Treatment for Ulcerative Jejunitis•Steroids •Immunosuppressive agents Azathioprine
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Coeliac Disease (Gluten Sensitive Enteropathy)
Association with Coeliac Disease •Dermatitis herpetiformis – blistering
sub -epidermal eruption of skin associated with Coeliac disease. It is extremely itchy appears on forearm, knees, buttocks and back.
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Dermatitis herpetiformis at the forearm, elbow region and on the hand
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Thank you
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