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:
- Bao F, Bhagat G. Histopathology of celiac disease. Gastrointest Endosc Clin N Am. 2012 Oct;22(4):679-94. [Medline]
- Oberhuber G. Histopathology of celiac disease. Biomed Pharmacother. 2000 Aug;54(7):368-72. [Medline]
- Dickson BC, Streutker CJ, Chetty R. Coeliac disease: an update for pathologists. J Clin Pathol. 2006 Oct;59(10):1008-16. [Medline]
- 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]
- Corazza GR, Villanacci V. Coeliac disease. J Clin Pathol. 2005 Jun;58(6):573-4. [Medline]
Created Aug 30, 2013.
