Celiac Disease: Celiac Sprue, Gluten-sensitive Enteropathy

What is Celiac Disease?Celiac Disease (CD) or gluten-sensitive enteropathy is a T-cell mediated disease occurring in genetically susceptible individuals induced by the ingestion of one of several proteins found in wheat (gliadins), barley (hordeins) and rye (secalinin).Symptoms classically include episodic diarrhea, abdominal pain and distention and weight loss.Approximately one half of adults develop clinically significant diarrhea.Interestingly, only one half of all patients with CD have symptoms referable to the gastrointestinal tract. What is the most common presentation of CD?The most common clinical presentation is iron-deficiency anemia due to both iron malabsorption and occult gastrointestinal bleeding.CD should be considered in any patient presenting with unexplained iron deficiency anemia, even in the absence of gastrointestinal symptoms.Involvement of the small bowel can lead to watersoluble (B12, folic acid) and fat-soluble (A, D, K, E) vitamin malnutrition and calcium deficiency.Thus patients may go on to develop such symptoms as peripheral neuropathy, osteoporosis, ataxia and coagulopathy.Infants with CD typically present with impaired growth, diarrhea and abdominal distention.Older children with severe, untreated CD may develop short stature, pubertal delays, rickets, dental enamel defects, behavioral disturbances and poor school performance.CD is associated with the human leukocyte antigen HLA-DQ2 in 90% to 95% of cases and HLA-DQ8 in the remaining 5% to 10% of cases. What is the diagnostic study of choice in CD?The definitive diagnosis of CD is made by fulfilling the triad of (1) a positive serologic test, (2) histologic findings of small-bowel biopsy specimens and (3) favorable clinical and serological response following a gluten-free diet.Histologic findings, suggestive of, but not pathognomonic for celiac's disease, include disruption of the normal villous morphology with villous atrophy and elongated crypts, decreased epithelial surface cell height, and increased lymphocytic infiltration into the mucosa.Serologic markers are used to screen patients with suspected CD and/or monitor their response and adherence to treatment with a gluten-free diet.Serological studies currently in clinical use include IgA endomysial antibody (IgA-EMA) and IgA tissue transglutaminase antibody (IgA-tTG).Antigliadin antibodies IgA and IgG (AGA) have moderate sensitivity but are less specific compared to IgA-EMA 71