Celiac Disease Flashcards
What is celiac disease?
autoimmune mediated intolerance to gliadin (a component of gluten) with a strong association to HLA-DQ2 and/or DQ8 haplotypes.
What makes celiac disease so unique?
both an environmental trigger (gluten) and the autoantigen (tissue transglutaminase).
- elimination of the environmental trigger leads to a complete resolution of the disease
For what does celiac disease increase your risk?
enteropathy-associated T-cell lymphoma
What is the pathogenesis of celiac disease?
- gliadin interacts with a HLA receptor on an antigen presenting cell.
- tissue tranglutaminase converts glutamine residues to glutamic acid.
- T helper cells are activated and acitivate B and killer T cells.
- plasma cell antibodies bind to gliadin bound to enterocytes and tissue transglutaminase.
- T cells release inappropriate inflammatory cytokines as well as inflict tissue damage.
What will you see on biopsy of small bowel with celiac disease?
blunting of intestinal villi with crypt openings
*** What is the Marsh criteria of intestinal lesions in celiac disease?
- type 0= pre-infiltrative (increased intraepithelial lymphocytes; IELs).
- type 1= infiltrative (more IELs).
- type 2= hyperplastic (edema of small bowel).
- type 3= destructive (flat mucosa with total mucosal atrophy and total loss of villi with crypt hyperplasia).
- type 4= hypoplastic (seen in T cell lymphoma).
In what group of people does celiac disease most commonly affect?
northern European ancestry
What are some risk factors or associations of celiac disease?
- dermatitis herpetiformis
- first-degree relative with celiac disease
- autoimmune thyroid disease
- down syndrome
- turner’s syndrome
- T1DM
** What is classical sprue?
villous atrophy leading to symptoms of malabsorption:
- steatorrhea
- weight loss
- nutrient or vitamin deficiency
- resolution will occur upon withdrawal of gluten-containing foods.
- will possess antibodies against gliadin and tissue transglutaminase.
** What is silent sprue?
- antibodies against gliadin or tissue transglutaminase.
- characteristic architectural remodeling of the mucosa seen in celiac disease.
- they DO NOT show clinical symptoms.
** What is latent sprue?
- celiac disease was present before, usually in childhood; the patient recovered completely with a gluten-free diet, remaining “silent” even when a normal diet was reintroduced.
- asymptomatic with NORMAL villous architecture
- must follow these pts
When does celiac disease normally present?
- ages 10-40
What are the signs and symptoms of celiac disease?
- common= diarrhea, fatigue, abdominal pain, weight loss, abdominal distention, and flatulence.
- uncommon (these push you more toward celiac disease)= vitamin D deficiency (osteopenia/osteoporosis), abnormal liver function tests (elevated transaminases), vomiting, iron-deficiency anemia, vit B12 deficiency, neurologic dysfunction, constipation, nausea.
** What is the classic disease triad of celiac disease?
- villous atrophy
- malabsorption (steatorrhea, wt loss, vitamin def).
- resolution of above with withdrawal of gluten
What are some non-GI clinical manifestations of celiac disease?
- infertility
- rheumatic
- osteoporosis
- neuro (depression, epilepsy, anxiety…)
- dermatitis herpetiformis
- T1DM
- selective IgA defieincy
- down’s syndrome
- liver disease
- pancreatitis
