Celiac Disease (Gluten-Induced Enteropathy): Classification

Oslo classification of celiac disease (adapted from).

Progress form Malabsorption syndrome Non-specific symptoms Celiac disease-specific Ak, tTG-Ak HLA-DQ2/-DQ8 Marsh stage 2 or 3
Classic + +/- + + +
Symptomatic – + + + +
Subclinical – – + + +
Refractory(Adults only) + +/- + + +
Potential – – + + –

Legend

  • Ac: antibody
  • TTG-Ak: transglutaminase antibody
  • HLA: human leukocyte antigen
  • Marsh stage: histological classification of celiac disease:
    • Marsh 2: ≥ 40 intraepithelial lymphocytes / 100 enterocytes (cells of the intestinal mucosa) + crypt hyperplasia.
    • Marsh 3: ≥ 40 intraepithelial lymphocytes / 100 enterocytes + crypt hyperplasia + villous atrophy.

Marsh classification

Type 0 Type 1 Type 2
Intraepithelial lymphocytes(threshold > 40 IEL/100). < 40/100 > 40/100 > 40/100
Crypts normal normal hypertrophic
Villi normal normal normal
Diagnosis
  • Only in cases of known celiac disease and histological
    Full remission (complete regression)
  • completely normal mucosa
  • Infiltrative type
  • Potential celiac disease
  • Must be further clarified, ie serology, possibly histology (fine tissue examination) after gluten exposure.
  • Hyperplastic type
  • Mostly “patchy lesions” at endoscopy in stages also type 1 or type 3 lesions
Type 3a Type 3b Type 3c Type 4
> 40/100 > 40/100 > 40/100 < 40/100
hyperplastic hyperplastic hyperplastic normal
slight atrophy subtotal atrophy total atrophy total atrophy
  • Typical histological picture of celiac disease.
  • Destructive type
  • 3b – high-grade villous plumping (only small villous remnants detectable).
  • 3c – flat mucosa
  • For celiac disease diagnostic stage !!!
  • Extremely rare, formerly probably a consequence of high-grade malnutrition in patients with “overlooked celiac disease”
  • Hypo-plastic type
  • No appreciable infiltration of the lamina propria (a layer of connective tissue found under epithelia).