celiac disease Flashcards
Pathogenesis of celiac disease
Gluten is a protein found in Wheat, barley, rye, Oats (sometimes)
Gluten is broken into gliadin, taken up by tissue transglutaminase (tTG)–> deamidated gliadin
Deaminated gliadin is taken up by APCs and presented to T cells make IFN y, T cells recruit B cells/plasma cells to make Anti-gliadin Anti endomysium (Anti tTG)
Serology of Celiac disea
Best test- IgA tissue transglutaminase, IgA Endomysial antibody (EMA), IgA and IgG Deamidated Gliading Antibodies
Anti Gliadin Antibodies that doent help- IGA level
Following positive serology–>
Endoscopy, Scalloping/ Notching of small bowl folds
Villous blunting/ atrophy
Small intestine biopsy- multiple biopsies should be obtained (6-8_ in the 2nd and 3rd portions of the duodenum
Gluten challenge- historically required for diagnosis, currently of little value, reserved for pts with self imposed GFD
Serologic tests (pit falls)
False negatives for 2 reasons: Ab levels fall on a gluten free diet: within days in very mild disease, usually takes 6-8 weeks, as long as a year in severe disease
Celiac patient that is IGA deficient, make IGA tTG and EMA, falsely negative, IGG Abs useful in situation, IgG deaminated GLiadin antibody best
Intestinal biopsy despite antibodies
Lab abnormalities of celiac disease
unexplained iron deficiency anemia, reduced serum albumin, folate and b12 def, elevated LFTs
Most powerful test- IgA endo mysial antibody, IgA Tissue transglutaminase!!!!!!!!!tTG!!!!!!!!, iga and Igg deamidated gliadin antibodies
anti gliadin antibodies is non specific
osteopenia and osteoporosis
Most common non GI presentation of CD
Osteo penia- untreated CD
Osteoporosis-
give a bone density scan
Non Gi presentation of celiac disease
type 1 DM, thyroid dysfunction, addisons, primary biliary cholangitis, IBD , autoimmune hepatitis
Downs and turner syndrome
IgA deficiency
Dermatitis herpetiformis- very pruritic disease
Gluten free diet
Neurologic symptoms
Gynocologic-
serologic pitfalls
False negatives for 2 reasons,
Ab levels fall on a gluten free diet, within days in very mild disease, usually takes 6-8 eks, as long as a year in severe disease
Celiac pts that is IGA deficient makes iga tTg and EMA falsely negative, Igg abs is useful, iGG deamidated dliadin Ab, intestinal biopsy
Pathology pit fallss
villous atrophy may be patchy (sometimes not all the biposy is taken)
Patient was on a GFD, can normalize, not all villous atrophy is celiac- NSAIDS, infections, crohns disease
Genetics
DQ2 ad DQ8 positive (not always)
Treatment
gluten free diet
all labels
gluten in medications
can have small amounts of gluten (20 ppm)
Increased risk of mortality
Malignant complications of Celiac disease
Enteropathy Associated T cell lymphoma- High grade T cell NHL, bad disease, rare disease but less raer in celiac pop
Risk normalizes on a GFD
Tropical sprue aka environmental enteropathy
syndrome of stunted growth and diarrhea that is common in developing countries
Affects>150 million children world wide, malnutrition contributes to path of disorder, but supplementary feeding and vitamin supplementation are able to reverse the syndrome
wheat allergy
similiar to celiac but also IgE allergic systemic causes
anaphylaxis
non celiac gluten senstitiviety
patient driven disease
13%
AAs have more
Athletes
Autism, ADD, Ms schizophrenia
Wheat is high to our diet
will not have any of the classic GI celiac serology
maybe fructan?