Using Marsh Scores to Diagnose Celiac Disease

The Marsh Score system was developed to classify celiac disease stages. It's a quick and easy-to-understand way to rate the damage to your small intestine caused by the disease.

If you're being tested for celiac disease, your healthcare provider 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.

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. Celiac disease stages range from 0 to 3, with three different subtypes of stage 3.

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.

This article walks you through the various stages of the Marsh Classification system and explains the intestinal lining changes that occur at each stage.

Stage 0

normal intestinal lining — 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 (a type of white blood cell) have not yet started to move into (infiltrate) the intestinal lining.

Stage 1

celiac disease Marsh Score 1 intestinal lining — © 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 among them than normal. The presence of too many lymphocytes indicates inflammation and the potential for damage.

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 painkillers 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.

Stage 2

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."

Larger-than-normal crypts are called "hyperplastic." 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 rash can appear anywhere on the body, but is most commonly seen on the extensor surfaces of the knees, elbows, shoulders, buttocks, sacral region, neck, face, and scalp.

Stage 3

image of marsh score 3 intestinal lining - celiac disease — © Ludvigsson et al, 2009

Most healthcare providers won't diagnose celiac disease until your intestinal lining Marsh Score 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:

Mild villous atrophy (Stage 3a): Your intestinal villi are still there, but are slightly smaller.
Marked villous atrophy (Stage 3b): Your intestinal villi have shrunken significantly.
Complete villous atrophy (Stage 3c): Your intestinal lining is totally affected. It is basically flat with no intestinal villi left.

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

The original Marsh Classification included stage 4, but this stage has since been removed to allow for greater consistency and reproducibility when pathologists are examining the tissue and creating their reports.

Summary

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 healthcare provider about what it means.

The gluten-free diet is the only treatment for celiac disease. After you start going gluten-free, your symptoms should improve within a few days, and with time, even bad intestinal damage will completely heal. By managing your symptoms, you can still lead a full life with celiac disease.

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