Modified Marsh Classification of Histologic Findings in Celiac Disease (Oberhuber)

Celiac disease is a systemic immune-mediated disorder triggered by dietary gluten in genetically susceptible persons. Gluten is a protein complex found in wheat, rye, and barley. Celiac disease is characterized by a broad range of clinical presentations, a specific serum autoantibody response, and variable damage to the small intestinal mucosa.

Modified Marsh Classification of histologic findings in celiac disease (Oberhuber)

Marsh Type IEL / 100 enterocytes – jejunum IEL / 100 enterocytes – duodenum Crypt hyperplasia Villi
0 <40 <30 Normal Normal
1 >40 >30 Normal Normal
2 >40 >30 Increased Normal
3a >40 >30 Increased Mild atrophy
3b >40 >30 Increased Marked atrophy
3c >40 >30 Increased Complete atrophy

IEL/100 enterocytes, intraepithelial lymphocytes per 100 enterocytes

  • Type 0: Normal; celiac disease highly unlikely.
  • Type 1: Seen in patients on gluten free diet (suggesting minimal amounts of gluten or gliadin are being ingested); patients with dermatitis herpetiformis; family members of celiac disease patients, not specific, may be seen in infections.
  • Type 2: Very rare, seen occasionally in dermatitis herpetiformis.
  • Type 3: Spectrum of changes seen in symptomatic celiac disease.

Simplified system (Corazza, Roberts, Ensari)

  • Grade A/Type 1: increased intraepithelial lymphocytes but no villous atrophy
    • Seen in patients on gluten free diet (suggesting minimal amounts of gluten or gliadin are being ingested); patients with dermatitis herpetiformis; family members of celiac disease patients, not specific, may be seen in infections
  • Grade B1/Type 2: villi still present but shortened
    • Spectrum of changes seen in symptomatic celiac disease
  • Grade B2/Type 3: complete villous atrophy
    • Spectrum of changes seen in symptomatic celiac disease

 

 

References:

  1. Bao F, Bhagat G. Histopathology of celiac disease. Gastrointest Endosc Clin N Am. 2012 Oct;22(4):679-94. [Medline]
  2. Oberhuber G. Histopathology of celiac disease. Biomed Pharmacother. 2000 Aug;54(7):368-72. [Medline]
  3. Dickson BC, Streutker CJ, Chetty R. Coeliac disease: an update for pathologists. J Clin Pathol. 2006 Oct;59(10):1008-16. [Medline]
  4. Oberhuber G, Granditsch G, Vogelsang H. The histopathology of coeliac disease: time for a standardized report scheme for pathologists. Eur J Gastroenterol Hepatol. 1999 Oct;11(10):1185-94. [Medline]
  5. Corazza GR, Villanacci V. Coeliac disease. J Clin Pathol. 2005 Jun;58(6):573-4. [Medline]

Created Aug 30, 2013.

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