- a.k.a gluten sensitive enteropathy
- 95% HLA DQ2 and HLA DQ8
- prevalence : 1 in 300-1500 (more common in Irish)
- occurs at any age (peaks in infancy and 50-60)
- presents with steatorrhea, diarrhea, abdominal pain, bloating, aphthous ulcer, weight loss, iron deficiency anemia, osteomalacia, failure to thrive
- associated with dermatitis herpetiformis
- diagnosis: IgA endomysial antibodies , alpha-gliadin antibodies or anti-transglutaminase antibodies (IgG if IgA deficient - 10% of Coeliac disease)
- duodenal/jejunal biopsy (gold standard): subtotal villous atrophy, hypertrophied crypts of Lieberkuhn, increase in intraepithelial lymphocytes (biopsy reverse/improvement with a gluten free diet)
- treatment: lifelong gluten free diet (wheat, barley, rye, oats)
- complications: risk of malignancy (esophageal, gastric, small bowel), anemia, hyposplenism, lactose intolerance, GI T cell lymphoma (suspect if refractory symptom or weight loss)
2 comments:
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