Refractory Celiac Disease Characteristics

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Healthcare providers diagnose refractory celiac disease when your small intestine fails to heal, despite a careful gluten-free diet. Here's more information about the condition, its diagnosis, and your treatment options.

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Most people diagnosed with celiac disease—probably around 90 percent—find relief from their symptoms, and their intestines begin to heal within a few weeks of starting the gluten-free diet.

However, some patients don't see that relief or find that their celiac disease symptoms recur after having been in abeyance for a while. If this is the case for you, your healthcare provider may mention the possibility of refractory celiac disease.

Refractory celiac disease occurs when your small intestine fails to heal and you continue to suffer from villous atrophy, even though you've been following a strict, careful gluten-free diet for a year or more. People with true refractory celiac disease—which is a very rare condition—are at much higher risk for serious complications, including a form of non-Hodgkin lymphoma associated with celiac disease.


This may sound scary, but don't let it frighten you: you probably don't have refractory celiac disease. Your odds of developing the condition are actually quite slim: one study found that only 1.5 percent of all celiac disease patients developed refractory celiac disease. And for most people who experience ongoing symptoms, there's another cause—usually, it's microscopic amounts of gluten in your diet that are causing your continuing symptoms. In a few cases, your healthcare provider may even find that you don't really have celiac disease after all.

Regardless of the odds of refractory celiac disease, if you're strictly adhering to the diet and find that you're still not feeling better, your healthcare provider will likely want to investigate further to find the problem. And if you do wind up with a diagnosis of refractory celiac, there are treatments that may help you heal.


Refractory celiac disease patients share several common characteristics: most are middle-aged or older (the condition is almost never seen in children), are women who have lost weight, and experience diarrhea.

In addition to their ongoing symptoms, most people with refractory celiac have malnutrition and vitamin deficiencies that indicate severe malabsorption.

However, other conditions can cause these same symptoms. The first step in diagnosing refractory celiac disease (or, more likely, ruling out the condition) will be for your healthcare provider to determine whether your celiac disease testing and diagnosis were correct in the first place.


To be properly diagnosed with celiac disease, you must have an endoscopy with a biopsy that shows villous atrophy: damage to the tiny villi in your small intestine that help you digest your food. Although they're uncommon, there are other conditions that also can cause similar intestinal damage.

To back up your original diagnosis, your healthcare provider may want to perform repeat celiac blood tests and a test to see if you have one of the celiac disease genes. If you definitely do have celiac disease, the next step in the investigation will be to determine whether you're following the gluten-free diet strictly. That could involve a meeting with a dietitian skilled in the intricacies of the diet.

Frankly, most people overestimate how well they follow the diet. For example, patients who continued to have symptoms following their diagnosis were still consuming some gluten, even though they believed they understood the diet and how to spot the hidden gluten in food products. Common slip-ups include missing gluten on food labels, eating out too often, and eating too many processed foods with poor labeling.

It's quite possible that a "reboot" of your diet can help solve any problems with continuing symptoms. But if it doesn't, then your healthcare provider should look for other potential causes, including lactose malabsorption, small intestine bacterial overgrowth, pancreatic insufficiency, and irritable bowel syndrome.

Once these other potential causes of ongoing symptoms have been ruled out, then your healthcare provider may consider a diagnosis of refractory celiac disease. At some celiac disease centers, specialists are using capsule endoscopy to view the entire small intestine and confirm the diagnosis.


If you've been diagnosed with refractory celiac disease, you've been told you have one of two types: type I or type II. Your type is determined by special analyses of a particular white blood cell, known as a T-cell, present in your small intestine.

People with type I have normal T-cells in their intestinal lining, while people with type II have abnormal T-cells. Type I is more common than type II: in one study, only 1 in 200 of all refractory celiac disease patients had the type II form of the condition.

In addition to being rarer, type II is the more dangerous form of refractory celiac disease: it doesn't always respond well to treatment, and it's the most likely to lead to non-Hodgkin lymphoma. In fact, clinicians consider it to be a low-grade form of lymphoma (one with a poor prognosis).

A Word From Verywell

Since true refractory celiac disease is so rare, patients who've been diagnosed with either form will most likely be referred to a celiac center with experience treating refractory forms of the disease. There, clinicians have several different approaches they can try to curb the progression of the disease and heal your symptoms.

7 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
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Additional Reading

By Jane Anderson
Jane Anderson is a medical journalist and an expert in celiac disease, gluten sensitivity, and the gluten-free diet.