What are the morphologic features of celiac disease?
The normal duodenal mucosa has numerous fingerlike projections, or villi, whereas in celiac disease the normal villous architecture is lost (blunted villi and crypt hyperplasia) and intraepithelial lymphocytes (IELs) are increased. Increased IELs are seen more toward the tips of the villi. These are T lymphocytes that can be highlighted by CD3 immunohistochemical stain.
The Marsh criteria represent a morphologic classification that defines the many histologic features of this entity. The modified classification (Marsh-Oberhuber) subdivides Marsh 3 into A, B, and C as partial, subtotal, or total villous atrophy, respectively. Corazza classification simplifies it further into Grade A, B1, and B2, representing Marsh type 1, 3a, and 3c, respectively. The comparison and summary of histologic classifications is depicted in the below table.
Histologic Classifications of Celiac Disease
|Marsh Modified (Oberhuber)||Histologic Criteria||Corazza|
|IEL *||Crypt Hyperplasia||Villous Atrophy|
|Type 1||Yes||No||No||Grade A|
|Type 3a||Yes||Yes||Yes (partial)||Grade B1|
|Type 3b||Yes||Yes||Yes (subtotal)|
|Type 3c||Yes||Yes||Yes (total)||Grade B2|
IEL, Intraepithelial lymphocytes.
* > 40 IEL per 100 enterocytes for Marsh modified (Oberhuber); > 25 IEL per 100 enterocytes for Corazza.
Treated celiac disease may show normal villous architecture but the IELs are still increased.
Adapted from Rubio-Tapia A, et al. ACG clinical guidelines: Diagnosis and management of celiac disease. Am J Gastroenterol 2013;108(5):656–676.