Celiac Disease Diagnosis: How Are Marsh Scores Used?

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Stages of Intestinal Damage Caused by Gluten

normal intestinal villi
This illustration shows what normal intestinal villi look like. Royalty Stock Photo/Science Photo Library/Getty Images

If you're being tested for celiac disease, your doctor likely will want to perform an endoscopy and a biopsy to see if your small intestine has been damaged by the gluten protein (found in the grains wheat, barley and rye). She will use a medical instrument called an endoscope with a tiny camera to look directly at your upper digestive system, and she will take very small samples of tissue from the lining of your small intestine.

Following this medical procedure, a pathologist will look at the small tissue samples to see if there's gluten-related damage. That pathologist will rate the condition of these samples based on a rating system called the Marsh Score. Your Marsh Score will determine whether you're diagnosed with celiac disease, and how bad your condition actually is.

More details about each stage, and actual biopsy samples showing varying extents of injury, appear on the pages that follow (use the links below or the arrows above to navigate through the photographs and descriptions). Here are brief descriptions:

Stage 0

The intestinal lining is normal, so celiac disease is unlikely.

Stage 1

Lymphocytes (a type of white blood cell that helps to protect you from disease) have started to move into the surface of the intestinal lining.

Stage 2

Even more lymphocytes appear in the intestinal lining, and the lining itself starts to look abnormal, with larger-than-normal depressions between the intestinal villi (the tiny finger-like projections of cells that do the hard work of absorbing nutrients from your food).

Stage 3

Stage 3 includes the Stage 2 changes (large numbers of lymphocytes, plus larger-than-normal depressions between the villi). In addition, in Stage 3 the villi themselves are starting to shrink and flatten. This process is called villous atrophy.

There are three subsets of Stage 3:

  • partial villous atrophy (Stage 3a)
  • subtotal villous atrophy (Stage 3b)
  • total villous atrophy (Stage 3c)

Stage 4

The villi are totally atrophied (completely flattened), and the depressions between them have shrunk, too.

As you'll learn on the following pages, celiac disease is not the only condition that can cause some of these changes. That's why a biopsy is only one of the diagnostic tests recommended for celiac disease — although a biopsy is considered "the gold standard" for celiac diagnosis, the results of celiac disease blood tests and the person's ultimate response to the gluten-free diet also can assist in getting the right diagnosis.

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Marsh Score for Celiac Disease, Stage 0

normal intestinal lining
This intestinal lining is normal - Marsh Score 0. Science Picture Co./Getty Images

This is a photograph taken during an endoscopy, showing a normal small intestine lined with healthy villi. 

When the tissue sample comes from a small intestine that looks like this, celiac disease is very unlikely. In this case, the biopsy sample would be Marsh Stage 0, also known as the "pre-infiltrative stage" (it's called "pre-infiltrative" because lymphocytes have not yet started move into, or "infiltrate," the intestinal lining).

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Marsh Score for Celiac Disease, Stages 1-2

celiac disease Marsh Score 1 intestinal lining
Intestinal lining - Marsh Score 1. © Ludvigsson et al, 2009

In celiac disease with a Marsh Score of Stage 1, the cells on the surface of the intestinal lining (known as the epithelial cells) have more lymphocytes (white blood cells) among them than normal.

In a normal small intestine, there shouldn't be any more than 30 lymphocytes per 100 epithelial cells, but in Marsh Score Stage 1, there are more than that. In the pathology report you should receive following your endoscopy and biopsy for celiac disease diagnosis, this would be called "increased intraepithelial lymphocytes."

Celiac disease isn't the only condition that can cause this increase in disease-fighting lymphocytes — you may see more lymphocytes than normal in your small intestinal lining in a variety of other conditions, including inflammatory bowel disease, Sjogren's syndrome, and other food intolerances. Infections with a bacteria called Helicobacter pylori (which is linked to ulcers), and use of over-the-counter pain kills such as aspirin and ibuprofen, also can cause this. 

People with celiac disease who are following the gluten-free diet, close family members of people with celiac disease, and people with dermatitis herpetiformis, an extremely itchy rash that's considered to be the skin manifestation of celiac disease, may have Marsh Score Stage 1 intestinal linings.

Celiac Disease: Marsh Score Stage 2

In Marsh Score Stage 2, you see more lymphocytes than normal (just as in Marsh Score Stage 1), and you also see bigger depressions than normal between the intestinal villi.

These depressions are called "crypts," and larger-than-normal crypts are called "hyperplastic," so if your pathology report following your biopsy says you have "hyperplastic crypts" or "crypt hyperplasia," it simply means the depressions seen in your biopsy are bigger than they would be in a normal intestinal lining.

Marsh Score Stage 2 is pretty rare — it's mostly seen in people who have dermatitis herpetiformis (which, as I said above, is the skin manifestation of celiac disease).

Acknowledgement:

This photomicrograph is from Figure 1b of Ludvigsson et al. BMC Gastroenterology 2009 9:19. doi:10.1186/1471-230X-9-19. The photomicrographs were obtained from Prof. Åke Öst, earlier chairman of the Swedish National Steering Group for Small Intestinal Pathology).

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Marsh Score for Celiac Disease, Stage 3

image of marsh score 3 intestinal lining - celiac disease
This shows intestinal lining at Marsh Score 3. © Ludvigsson et al, 2009

Most doctors won't diagnose celiac disease until your intestinal lining reaches Marsh Score Stage 3. In this stage, the changes of Stage 2 are present (more lymphocytes than normal and larger-than-normal depressions). In addition, in Marsh Score Stage 3 your intestinal villi are shrinking and getting flatter, a process known in medical terminology as "villous atrophy."

There are three sub-stages in Marsh Score Stage 3:

  • partial villous atrophy (Stage 3a), which means your intestinal villi are still there, only smaller
  • subtotal villous atrophy (Stage 3b), which means your intestinal villi have shrunken further, and
  • total villous atrophy (Stage 3c), which means your intestinal lining is basically flat, with no intestinal villi left.

Most people who are diagnosed with celiac disease are in Marsh Score Stage 3.

Acknowledgement:

This photomicrograph is from Figure 1c of Ludvigsson et al. BMC Gastroenterology 2009 9:19. doi:10.1186/1471-230X-9-19. The photomicrographs were obtained from Prof. Åke Öst, earlier chairman of the Swedish National Steering Group for Small Intestinal Pathology.

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Marsh Score for Celiac Disease, Stage 4

intestinal lining - celiac disease marsh score 4
This intestinal lining is at celiac disease Marsh Score 4. © Ludvigsson et al, 2009.

Celiac disease Marsh Score Stage 4 is the most advanced stage of the condition, and it fortunately isn't seen all that often. In Stage 4, your villi are totally flattened (atrophied) the depressions between them (the crypts) are shrunken, as well.

Some people who are elderly when they're diagnosed with celiac disease have biopsies that are classified as Marsh Score Stage 4. Those at Stage 4 may be at higher risk for celiac disease complications, including lymphoma.

(Edited by Jane Anderson)

Acknowledgement:

This photomicrograph is from Figure 1d of Ludvigsson et al. BMC Gastroenterology 2009 9:19. doi:10.1186/1471-230X-9-19. The photomicrographs were obtained from Prof. Åke Öst, earlier chairman of the Swedish National Steering Group for Small Intestinal Pathology.

Sources

Ludvigsson JF et al. Validation study of villous atrophy and small intestinal inflammation in Swedish biopsy registers. BMC Gastroenterology. 2009;9:19.

Snyder CL, Young DO, Green PHR, Taylor AK. GeneReviews: Celiac Disease. 2009

Hill ID et al. Guideline for the diagnosis and treatment of celiac disease in children: recommendations of the North American Society fo Pediatric Gastroenterology, Hepatology and Nutrition. Journal of Pediatric Gastroenterology and Nutrition. 2005;40:1-19.

Stanford Medicine Surgical Pathology Criteria: Celiac Disease. Accessed Jan. 30, 2016.

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