celiac sprue

celiac sprue:
                                       also known as  - GLUTEN SENSITIVE ENTEROPATHY
                                                                
                                                                - NON TROPICAL SPRUE


It is a chronic disease of the digestive tract that results in an inability to tolerate GLIADIN

  • It affects proximal small intestine mucosa (duodenum + jejunum)
  • completely reversible within 6 months of gluten restriction (clinical + histologically)
  • if not reversible known as REFRACTORY CELIAC DISEASE
  • it is also known as ICEBERG DISEASE
TYPES:
  1. CLASSICAL CELIAC DISEASE: All signs and symptoms of malabsorption
  2. ATYPICAL CELIAC DISEASE: Present with symptoms other than malabsorption
  3. SILENT CELIAC DISEASE : Serological positivity , no symptoms
  4. LATENT CELIAC DISEASE : genetically at risk of developing disease. serology -ve,no symptoms

 - HLA DQ2  OR HLA DQ8
 
CLINICAL FEATURE:



  1. Diarrhoea/weight loss
  2. abd distension/ascites
  3. foul smelling stools
  4. bone pain/fractures
  5. cutaneous manifestations

atypical celiac disease
  1. short stature and failure to thrive
  2. iron deficiency anemia
  3. osteopenia
  4. ataxia and peripheral neuropathy
  5. Hyposplenism (howell jolly bodies)
  6. increase LFT


Extraintestinal manifestations
  1. Dermatitis herpetiformis
  2. Type 1 DM
  3. Downs syndrome
  4. Autoimmune hypothyroidism/hyperthyroidism
  5. Hypersensitive pneumonitis/bird fanciers lung
  6. Ig A deficiency/Ig A nephropathy


Evaluation of celiac disease
serology:
- anti endomysial antibody (more specificity)
- anti tissue transglutaminase antibody (more sensitivity)

biopsy:

  • villous atrophy
  • crypt hyperplasia
  • maintained mucosal thickness
  • lamina propria infiltrates
  • vacuolar degeneration of surface epithelium
diagnosis of celiac is made only on the basis of serology + biopsy
  • Biopsy (+)   +   antibody(+)   = treat
  • Biopsy (-)   +   antibody(-)   = no celiac
  • Biopsy (-)   +   antibody(+)   = repeat biopsy
  • Biopsy (+)   +   antibody(-)   = no chance of celiac


treatment
  • Gluten restriction
  • Refractory celiac  -  steroids
3 potential dangerous complications
  1. enteropathy associated T - cell lymphoma
  2. small intestinal adenocarcinoma
  3. esophageal squamous cell carcinoma

can eat - rice,corn,maize,potato,soya bean

must not eat - wheat,barley,rye,oats



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