Pathogenesis and classification of celiac disease

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Type

Clinical features

Possible pathogenesis

Genetic factors (hyperlink image to directly above)

Gene abnormalities in HLA-DQ2 and/or DQ8

Serum autoantibodies (hyperlink image to directly above)

IgA antibodies to gliadin

IgA antibodies against endomysium

Antigen identified as tissue transglutaminase (tTG)

IgG antibodies against deamidated gluten peptides (DAG)

Gliadin reactive T cells (hyperlink image to directly above)

T cell cross reactivity to deamidated gluten peptides

Gliadin receptor on intestinal epithelial cells (hyperlink image to directly above)

May transport gliadin proteins to intestinal lamina propria and activate T cell response against villi

Classification

Symptomatic disease

- Classic celiac disease

- - Villous atrophy

- - Symptoms of malabsorption

- - Resolution of findings with gluten-free diet

- Atypical celiac disease

- - Minor GI symptoms

- - Villous atrophy on duodenal biopsy

- - Positive serological antibody tests

Asymptomatic or subclinical disease

- Mild clinical findings

- Usually diagnosed due to high clinical suspicion

Potential (latent) celiac disease

- Positive serological antibody tests

- Normal duodenal biopsies with possible increased intraepithelial lymphocytes

- No clinical symptoms or signs of malabsorption

Refractory celiac disease (gluten-free diet unresponsive)

- Possible causes of underlying malabsorption

- - Non-malignant small intestine inflammatory disorder

- - Semi-malignant inflammatory condition

- - Overt enteropathy-associated T cell lymphoma

- - Collagenous sprue

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Celiac disease

Pathogenesis and classification of celiac disease

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