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home - Small Bowel - Coeliac Disease - Coeliac Clinical Presentation Written by Dr Sebastian Zeki

Coeliac Clinical Presentation

Small bowel histology: Chronic inflammatory infiltrate in lamina propria. Lymphocytes in epithelium. Decreased epithelial cell surface height. Loss of height. Crypt hyperplasia. Written by Dr Sebastian Zeki Celiac intestinal lesion and symptoms not correlated. Upper jejunal mucosal immunopathology Atrophic hyperplastic Flat destructive Infiltrative Hyperplastic 2 Infiltrative 1 Pre-infiltrative 0 Histopathology Endoscopic Types:Marsh stage 0 which is normal mucosa.Marsh stage 1which is an increased number of intra-epithelial lymphocytes, usually exceeding 20 per 100 enterocytes.Marsh stage 2 whuch us a proliferation of the crypts of Lieberkuhn.Marsh stage 3 which is a partial or complete villous atrophy.Marsh stage 4 which is hypoplasia of the small bowel architecture. Autoimmune diseases (eg, Type 1 diabetes mellitus, collagen vascular disease, autoimmune thyroiditis) may reach > 30 % of patients diagnosed after age 20Risk Of MalignancyIncreased risk of Non-Hodgkin's lymphoma and GI cancers increased mdoer-atelyRisk of Non-Hodgkins continues for 5 years after diagnosisBreastand possibly lung cancer risk appears to be decreased. Non-GI Manifestations GI Manifestations Malabsorption symptoms:Growth failure in childrenWeight lossSevere anemiaNeurologic disorders from deficiencies of B vitaminsOsteopenia from deficiency of vitamin D and calcium FlatulenceMeteorism (due to colonic bacterial digestion of malabsorbed nutrients). Diarrhea with bulky, foul-smelling, floating stools due to steatorrhea Malabsorption Kidney diseaseGlomerular IgA deposition is common, occurring in as many as one-third of patients.Usually no clinical manifestation HyposplenismKnown association although pathology unknownProphylactic pneumococcal vaccination has been suggested. Metabolic bone diseaseCommon to have porosis/ peoniaCan occur in asymptomatics.Due to secondary hyperparathyroidism secondary to vit D deficiency.May not reach normality with gluten free diet Risk of fractures is only slightly increased . ArthritisArthritis in 41 % in patients on a regular diet to 22 % in those on a gluten-free diet .Can be peripheral or axial or both Histological Lesions:Type 0 lesion involves a mild alteration characterized by increased intraepithelial lymphocytes.Type 3 lesion involves a flat mucosa with total mucosal atrophy, complete loss of villi, enhanced epithelial apoptosis and crypt hyperplasia .Type 4 lesion are characteristic of T cell lymphomas.Type 4 lesions have the same histologic features seen in the type 3 lesion except that lamina propria hyperplasia becomes hypoplasia. Neuropsychiatric diseasePrimary manifestation of CD may be neuropsychiat-ric symptoms such as ataxia, depression, anxiety, or epilepsy .Up to 60% of idiopathic ataxia may have coeliacPathogenesis may be: Regional hypoperfusion or presence of IgA tissue transglutaminase deposition around cerebellar vessels. Coeliac Disease- Clinical manifestations Clinical ManifestationsAges 10-40 Subclinical diseaseCan exist in a very mild form and may go largely undetected.Severity of disease may be weakly correlated with the level of TTG IgA.Risk of malignancy with subclinical coeliac disease is not known- is lower if dont have malabsorption.Risk becomes normal on gluten-free diet.

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