What is the pathophysiology of celiac disease (sprue)?

Updated: Nov 29, 2019
  • Author: Stephan U Goebel, MD; Chief Editor: BS Anand, MD  more...
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Celiac disease has a strong hereditary component. The prevalence of the condition in first-degree relatives is approximately 10%.

A strong association exists between celiac disease and two human leukocyte antigen (HLA) haplotypes (DQ2 and DQ8). Damage to the small intestinal mucosa occurs with the presentation of gluten-derived peptide gliadin, consisting of 33 amino acids, by the HLA molecules to helper T cells. Helper T cells mediate the inflammatory response. Endogenous tissue transglutaminase deamidates gliadin into a negatively charged protein, increasing its immunogenicity. Autoantibodies to type 2 transglutaminase (TG2) is a hallmark of celiac disease. [4]  Absence of intestinal villi and lengthening of the intestinal crypts characterize the mucosal lesions in untreated celiac disease. More lymphocytes infiltrate the epithelium (intraepithelial lymphocytes). Destruction of the absorptive surface of the intestine leads to a maldigestive and malabsorption syndrome. [5]

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