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Pathology - Coeliac disease
Definition: An autoimmune condition resulting from an aberrant immunological response to dietary gluten.
Epidemiology • Prevalent, impacting approximately 1% of the population.
Aetiology: Dietary gluten and associated proteins. Pathogenesis The offending proteins are inadequately degraded by intestinal proteases. Intact peptides penetrate the lamina propria and undergo deamidation by tissue transglutaminase, resulting in a negative charge. Negatively charged peptides bind more efficiently to HLA receptors on antigen-presenting cells, which are detected by intestinal T lymphocytes. Activated T-cells elicit an immunological response in the gut wall.
Presentation • Symptoms associated with the gastrointestinal tract may include weight loss, stomach pain, and diarrhea. Many individuals, however, are asymptomatic and are detected solely during the study of iron deficiency anemia. Serology • The detection of serum IgA endomysial or transglutaminase antibodies is very specific and sensitive for celiac disease.
Macroscopy • Villi may exhibit blunting and flattening when observed under a dissecting microscope.
Histopathology • Advanced cases have elevated intraepithelial lymphocytes, many lymphocytes and plasma cells in the lamina propria, villous atrophy, and crypt hyperplasia. • Less severe cases may present solely with increased intraepithelial lymphocytes in the absence of villous atrophy. 2 It is important to recognize that these alterations are not exclusive to celiac disease; similar modifications can be observed in several other illnesses. Biopsy findings must be analyzed in conjunction with the clinical and serological context.
Prognosis: • Rigorous compliance with a gluten-free diet results in symptom relief and histological normalization. • Elevated risk of type 1 diabetes, autoimmune thyroid disease, dermatitis herpetiformis, oropharyngeal and esophageal cancer, small bowel adenocarcinoma, and a rare, although very aggressive, variant of T-cell lymphoma referred to as enteropathy-associated T-cell lymphoma
Definition: An autoimmune condition resulting from an aberrant immunological response to dietary gluten.
Epidemiology • Prevalent, impacting approximately 1% of the population.
Aetiology: Dietary gluten and associated proteins. Pathogenesis The offending proteins are inadequately degraded by intestinal proteases. Intact peptides penetrate the lamina propria and undergo deamidation by tissue transglutaminase, resulting in a negative charge. Negatively charged peptides bind more efficiently to HLA receptors on antigen-presenting cells, which are detected by intestinal T lymphocytes. Activated T-cells elicit an immunological response in the gut wall.
Presentation • Symptoms associated with the gastrointestinal tract may include weight loss, stomach pain, and diarrhea. Many individuals, however, are asymptomatic and are detected solely during the study of iron deficiency anemia. Serology • The detection of serum IgA endomysial or transglutaminase antibodies is very specific and sensitive for celiac disease.
Macroscopy • Villi may exhibit blunting and flattening when observed under a dissecting microscope.
Histopathology • Advanced cases have elevated intraepithelial lymphocytes, many lymphocytes and plasma cells in the lamina propria, villous atrophy, and crypt hyperplasia. • Less severe cases may present solely with increased intraepithelial lymphocytes in the absence of villous atrophy. 2 It is important to recognize that these alterations are not exclusive to celiac disease; similar modifications can be observed in several other illnesses. Biopsy findings must be analyzed in conjunction with the clinical and serological context.
Prognosis: • Rigorous compliance with a gluten-free diet results in symptom relief and histological normalization. • Elevated risk of type 1 diabetes, autoimmune thyroid disease, dermatitis herpetiformis, oropharyngeal and esophageal cancer, small bowel adenocarcinoma, and a rare, although very aggressive, variant of T-cell lymphoma referred to as enteropathy-associated T-cell lymphoma
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