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- An immune disorder triggered by an environmental agent (the gliadin component of gluten) in a genetically predisposed individual:
- Seen primarily in whites of northern European ancestry
- Celiac presentations:
- Celiac disease is classically a diarrhea illness characterized by villous atrophy with symptoms of malabsorption (steatorrhea, weight loss, vitamin deficiencies, anemia) with resolution of symptoms with a gluten-free diet.
- Patients only exhibit only minor GI symptoms, but note a myriad of extraintestinal symptoms (e.g., anemia, dental enamel defects, neurologic symptoms, infertility).
- Asymptomatic (silent) disease:
- Usually found when screening 1st-degree relatives.
- Positive genetics, without signs/symptoms.
- System(s) affected: Gastrointestinal
- Synonym(s): Sprue; Gluten enteropathy; Celiac sprue
- Disease primarily of individuals of Northern European ancestry
- Predominant sex: Female > Male (3:2)
- ~1/133–160 persons in the US (1)
- An estimated 3 million Americans have celiac disease.
- 1st-degree relatives: 10% incidence
- 71% in monozygotic twins
Homogenicity for HLA-DQ2 increases the risk of celiac disease and enteropathy associated T-Cell lymphoma.
Avoid all gluten-containing products (wheat, barley, rye, and possibly oat products).
Sensitivity to gluten, specifically gliadin fraction
Commonly Associated Conditions
- Dermatitis herpetiformis: Very strong association with celiac disease (2)
- May have secondary lactase deficiency
- Extraintestinal manifestation may include marked decrease in bone density.
- Autoimmune thyroiditis
- Diabetes, type 1: Prevalence of celiac disease in type 1 diabetes is 3–8%.
- Elevated AST and ALT
- Recurrent fetal loss/infertility
- Irritable bowel syndrome (IBS)
- Restless leg syndrome
- GI lymphoma: Celiac disease is associated with both Hodgkin and non-Hodgkin lymphomas:
- Particular increase in prevalence of T-cell non-Hodgkin lymphoma (3)
- Recent studies show the risk of lymphoproliferative malignancies in celiac disease is dependent on small intestinal histopathology.
- There appears to be little to no increased risk in latent celiac disease (seropositive, but normal biopsy) (4)[C].
- Migraine (more prevalent in patients with celiac disease) (5)
- Celiac disease may be an under appreciated cause of male and female infertility.
- Consider celiac disease in pregnant women with severe anemia.