Using Marsh Scores to Diagnose Celiac Disease

Marsh scores rate intestinal damage from celiac disease

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

Following your endoscopy, a pathologist will look at your 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, as well as how advanced your condition is.

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.

1
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. Since the intestinal lining appears normal, it's unlikely that the person has celiac disease.

In this case, the biopsy sample would be classified as 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.

2
Stages 1 and 2

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

A stage 1 Marsh score means 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 should be no more than 30 lymphocytes per 100 epithelial cells, but in cases deemed stage 1, there are more than that. If you have stage 1, your pathology report will likely read "increased intraepithelial lymphocytes."

Celiac disease isn't the only condition that can cause an increase in disease-fighting lymphocytes. You may have more lymphocytes if you have 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 also have a stage 1 Marsh score. 

With stage 2, you see more lymphocytes than normal as well as 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 means the depressions seen in your biopsy are bigger than they would be in a normal intestinal lining.

Stage 2 is pretty rare—it's mostly seen in people who have dermatitis herpetiformis.

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).

3
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 stage 3. In this stage, the changes of stage 2 are present––more lymphocytes than normal and larger-than-normal depressions––as well as shrinking and flattening intestinal villi, what is known as villous atrophy.

Stage 3 has three substages:

  • Partial villous atrophy (Stage 3a): Your intestinal villi are still there, but are smaller
  • Subtotal villous atrophy (Stage 3b): Your intestinal villi have shrunken significantly
  • Total villous atrophy (Stage 3c): Your intestinal lining is basically flat with no intestinal villi left

Most people who are diagnosed with celiac disease have a stage 3 Marsh score. 

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.

4
Stage 4

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

Stage 4 is the most advanced stage and 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.

Stage 4 is most common amongst older people with celiac disease. If your Marsh score is a stage 4, you may have a higher risk for celiac disease complications, including lymphoma.

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.

5
A Word from Verywell

Gastroenterologists use Marsh scores to quantify the damage to your small intestinal lining from celiac disease. Your Marsh score will determine whether you have celiac disease, and how much it has damaged your intestinal villi.

This guide to Marsh score levels can help you interpret the report from your intestinal biopsy. However, if you're having trouble understanding it, don't be afraid to talk to your doctor about what it means. And remember: once you start the gluten-free diet, even bad intestinal damage should begin to heal.

Sources:

Ludvigsson JF et al. Validation study of villous atrophy and small intestinal inflammation in Swedish biopsy registersBMC 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 NutritionJournal of Pediatric Gastroenterology and Nutrition. 2005;40:1-19.

Stanford Medicine Surgical Pathology Criteria: Celiac Disease.

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