Overview

• Definition: autoimmune disorder characterized by an intestinal hypersensitivity to gluten, a grain protein [1]

• Synonyms: celiac sprue; gluten-sensitive enteropathy

• Sex: ♀ > ♂

• Age of onset

  • The disease can occur at any age.

  • Peak incidence is bimodal:

    • At 8–12 months (or 2–3 months following the first exposure to gluten through diet containing wheat)

    • Third to fourth decade of life

• Prevalence: in the US ∼ 1:150 [2]

• Race: more common in individuals of northern European descent

• Genetic predisposition with association to HLA antigens [3]

  • HLA-DQ2 in 90–95% of patients

  • HLA-DQ8 in 5–10% of patients

• Consuming gliadin from grains such as wheat, rye, and barley leads to an autoimmune reaction within the small intestinal wall.

• Commonly associated with autoimmune diseases

Symptoms manifest when a genetically predisposed individual develops an immunological response to gliadin, an alcohol-soluble fraction of gluten.

• Consumption of food containing gluten → tissue transglutaminase is released → modifies gliadin from gluten proteins → pathogenic T cells react to and are activated by modified gliadin → mediate chronic intestinal inflammation → epithelial damage resulting in villous atrophy, crypt hyperplasia, and loss of brush border → impaired resorption of nutrients in the small intestine (especially in the distal duodenum and proximal jejunum) → malabsorption symptoms