Coeliac Disease

• Chronic small intestinal disease in which there is a characteristic mucosal lesion, impaired nutrient absorption and which improves on withdrawl of wheat gliadins and related grain proteins from the diet
• In Europe the prevalence is 0.5 to 1% of the population
• It is generally confined to those of white ethnic origin

Pathogenesis of Coeliac Disease

• Due to sensitivity to glutan which is the water soluble, alcohol soluble protein of wheat and closely related grains
• T cell mediated chronic immune response
• Within the small intestinal mucosa there are CD8 IEL and CD4 T cells within the LP
• Interestingly CD4 T cells recognise gliadin but the CD8 cells dont
• The recognised epitopes of gliadin are confined to residues 57 and 75 of gliadin
• Family history is important
• Almost all individuals are either HLA-DQ2 or HLA-DQ8 haplotype

Morphology of Coeliac Disease

• Diffuse enteritis with villus atrophy and crypt hyperplasia (elongated regenerative crypts)
• Surface epithelial damage with loss of the brush border and IEL
• LP is infiltrated with plasma cells, CD4 T cells, macrophages, eosinophils and mast cells
• Changes are more marked in the proximal small intestine
• Mucosal histology is reversed following a period of glutan exclusion from the diet

Clinical features of Coeliac Disease

• May present in infants up to middle age with diarrhoea, flatulence, weight loss and fatigue
• It is associated with blistering skin lesions, dermatitis herpetiformis and neurological disorders
• Diagnosis is favoured by detection of anti-gliadin or anti-endomysial antibodies
• Definitive diagnosis rests upon;
  • Clinical documentation of malabsorption
  • Demonstration of intestinal lesion on biopsy
  • Unequivocal improvement of symptoms and mucosal histology on gluten withdrawl from the diet

• There is a long term risk of malignancy;
  • Non-Hodgkin lymphoma
  • Small intestinal adenocarcinoma
  • Oesophageal squamous cell carcinoma