Syndrome of malabsorption. Gluten enteropathy Flashcards
what is celiac disease ?
it is a multifactorial autoimmune disorder that occurs in genetically susceptible individuals leading to malabsorption which is generally triggered by gluten
what is celiac disease triggered by ?
celiac disease only occurs after dietary exposure
gluten related prolamins such as wheat , rye secalinus and barley
gliadins
autoantigen
tissue transglutaminase
celiac disease primarily affects in what location ?
primarily affects the proximal small intestines (duodenum , jejunum , ileum)
the freq of celiac disease is rising due to what ?
changes in wheat production and prep
increased awareness of the disease
what are the risk factors for celiac disease ?
affected first degree relative
autoimmune: type 1 diabetes hashimoto thyroiditis IgA nephropathy arthritis
genetic
down syndrome
turner syndrome
williams syndrome
drugs - interferon alfa
bacteria and virus
infant feeding practices
how can you diagnose your genetic susceptibility to celiac disease ?
haplotype HLA -
90 percent express DQ2 heterodimer
7% DQ8
3% half of DQ2
celiac diseases are classified according to what clinical spectrum ?
active - DQ2/8 symptomatic postive biopsy and positive autoimmune serology
silent - DQ2/B aysmptomatic positive biopsy positive autoimmune serology
latent DQ2/8 symptomatic/ asymptomatic negative biopsy positive or negative autoimmune serology
potential DQ2/8 SYMPTOMATIC OR ASYMPTOMATIC negative biopsy posittive serology
what are the typical GI symptoms ?
chronic diarrhoea anorexia abdominal distension - bloating abdominal pain vomiting
delayed puberty
short
describe the signs of celiac crisis?
explosive watery diarrhoea makes abdominal distention - bloating dehydration hypotension lethargy severe hypokalaemia
what are the main extra intestinal manifestations of celiac disease ?
dermatitis herpetiformis
dental enamel hypoplasia
aphthos ulcers
delayed tooth eruption
iron def anemia
chronic hepatitis and hypetransaminasemia
type 1 DM
bone:
arthritis and arthralgia
osteoperosis
neurological - occipital calcifications ,
epilepsy , gluten induced ataxia
psychiatric disorders , seizures
sub or infertility
what are the serological markers for celiac disease ?
anti TG2 IgA or IgG (anti tissue tranglutaminase antibody ) for children over 2 years old!
antiendomysial antibody EMA -IgA
recommended because sensitivity and specific
these two not recommended because low sensitivity and specificity
anti deaminated gliadin peptide antibody - IgG/ IgA
anti gliadin antibody - IgG / IgA (for children under 2 years old)
what is the histopathological classification of celiac disease ?
MARSH CLASSIFICATION
0
intraepithelial lymphocytes less than 40
normal crypts
normal villi
1
more than 40
normal crypts and villi
2
more than 40
increased crypts
normal villi
3a
more than 40
increased crypts
mild atrophy
3b
more than 40
increased crypts
marked atrophy
3c
more than 40
increased crypts
villi absent
in which country is celiac disease ver hip
Finland almost affects 3 percent of the population
describe the pathoetiology of celiac disease
gluten in the small intestines causes the enterocytes to release the protein zonulin which loosens the tight junctions
and these gluten fragments cross the intestinal lining in abundance and accumulate
causing immune reaction of INTRAEPITHELIAL LYMPHOCYTES
tissue transglutaminase released by damaged cells modifies the gluten taken up by antigen presenting cells
t h cells kill the enterocytes
b cells release antibodiestargetting gluten and TTG
what is the current criteria for definitive diagnosis of Celiac disease?
both requirements are mandatory
villous atrophy with crypt hyperplasia and abnormal surface epithelium
clinical remission from withd r awal of gluten from diet
celiac antibodies - and their disappearance in gluten free diet = adds weight to diagnosis
HLA testing
