A small but frustrating percentage of people with celiac disease can’t seem to get better despite maintaining a vigilant gluten-free diet. In the past, these people were diagnosed with refractory celiac disease (RCD). Today they will be diagnosed with non-responsive celiac disease (NRCD), and a smaller subset of these individuals will be diagnosed with refractory celiac disease (RCD).
The hopeful change here represents an opportunity for some people previously diagnosed as having RCD to be updated as NRCD patients instead. This is hopeful because a majority of these individuals can heal by implementing a newly developed diet, the Gluten Contamination Elimination Diet, for only 3 to 6 months.
This lesson will define non-responsive celiac disease and the two types of refractory celiac disease, then discuss the most effective treatments for these frustrating conditions.
Table of Contents:
- What Is Non-Responsive Celiac Disease?
- Why So Urgent? New Research on Cancer and Celiac Disease
- The Eye-Opening New Research: Surprising Amounts of Gluten Cross-Contamination
- Type 1 Refractory Celiac Disease
- Type 2 Refractory Celiac Disease
- Gluten Contamination Elimination Diet
- Immunosuppressive Medications
- Conclusion and Review
- Take The Quiz
What Is Non-Responsive Celiac Disease?
A vast majority of people diagnosed with celiac disease respond well to a strict gluten-free diet. For those people, within just a couple of months a gluten-free diet soothes the inflammation along the lining of their small intestine and allows their villi to make a dramatic recovery.
Note that I specified *strict* gluten-free diet. You can’t cheat; you must be vigilant in converting to a strict gluten-free lifestyle.
But around 10% of celiac patients don’t respond to a gluten-free diet. When patients still suffer from medically significant villous atrophy 6 to 12 months into adhering to a strict gluten-free diet, they are now diagnosed as having non-responsive celiac disease.
In the past, these patients were diagnosed as having Refractory Celiac Disease. In fact, depending on how well your doctor stays up to date, you could still be treated this way.
Now if you are diagnosed with NRCD, you should be placed on the Gluten Contamination Elimination Diet (GCED) for 3 to 6 months. A majority of people placed on this diet will heal and can then return to a normal gluten-free diet. Anyone who does not respond to the GCED diet will then be further examined and diagnosed as having type 1 or type 2 refractory celiac disease.
Why So Urgent? New Research on Cancer and Celiac Disease
Research just published in the Annals of Internal Medicine indicates that when people don’t implement a strict and consistent gluten-free diet, they greatly increase their risk for cancer. The study is entitled, “Mucosal Healing and Risk for Lymphoproliferative Malignancy in Celiac Disease: A Population-Based Cohort Study”. You can read more about this study here:
Poor Treatment of Celiac Disease May Lead to Cancer. To learn more about the strong relationship between cancer and celiac disease, read this new Gluten Intolerance School lesson: Celiac Disease and Cancer
The New Eye-Opening Research
A recent study illuminated how many patients previously diagnosed as having refractory celiac disease can heal and return to a normal gluten-free diet after adhering to a specific, highly restrictive short-term elimination diet.
In a study published in the medical journal BMC Gastroenterology in February, 2013, celiac researchers hypothesized and confirmed that many patients previously diagnosed as having RCD were actually highly sensitive patients reacting to tiny amounts of gluten contamination in gluten-free grains (and possibly other contaminated GF foods).
To understand how they discovered this phenomenon, we must first understand a study published in the June, 2010, issue of the Journal of the American Dietetics Association. The researchers in that study tested a wide selection of inherently gluten-free grains for gluten cross-contamination. For example, they tested grains and grain flours like millet and sorghum.
Unfortunately, they discovered that 41% of these grains contained small traces of gluten, and a full 32% of them contained amounts in excess of the accepted safe limit of 20 parts-per-million.
It’s important to note that all the products tested were not explicitly labeled gluten-free. The manufacturers, however, still presented their products as inherently gluten-free (which, if it weren’t for cross contamination, would technically be true).
For the BMC Gastroenterology study, the researchers hypothesized that this previously unknown cross-contamination may explain at least some of the cases of non-responsive celiac disease. In other words, they hypothesized these patients didn’t actually have refractory celiac disease, but rather weren’t healing because of unsuspected gluten cross-contamination.
In fact, 82% of the patients in the study responded well to the Gluten Contamination Elimination Diet, which appears to prove their hypothesis. In addition, these patients appeared to maintain their health even after re-including all the previously avoided foods. This suggests that once their immune systems recuperated from the cross-contamination, they could handle those small traces of gluten without issue.
Note: I’m personally interested in seeing how these patients do long-term. Will any of them experience relapsed symptoms after a year or so and need to go back on the strict GCED diet? And what about peripheral conditions or symptoms beyond gastrointestinal symptoms and small intestine health?
I also would have liked to see the researchers slowly re-introduce tested selections of those grains. In other words, test some millet or sorghum to make certain it is gluten-free, then slowly re-introduce it. This would help determine if any of them were experiencing not just cross-contamination but a reaction resulting from protein cross-reactivity.
Type 1 Refractory Celiac Disease
Type 1 refractory celiac disease (RCD1) is present when a celiac disease patient does not respond to both a strict gluten-free diet and to the Gluten Contamination Elimination Diet.
In addition, RCD1 patients are excluded from having type 2 refractory celiac disease (RCD2) by determining that symptoms and test results are influenced by the presence of other bowel ailments, such as Crohn’s disease, fructose intolerance, intestinal lymphoma, lactose intolerance, microscopic colitis, pancreatic insufficiency, small bowel bacterial overgrowth, or ulcerative colitis.
Please note that you should let a doctor diagnose this condition and don’t assume you have it just because you don’t feel well even after being gluten-free diet for a while.
A doctor will need to make certain gluten hasn’t slipped into your life somehow and then make sure your bowel really isn’t healing, usually through a colonoscopy with biopsy or a esophagogastroduodenoscopy (EGD).
Type 2 Refractory Celiac Disease
To be diagnosed with type 2 refractory celiac disease (which is very rare), first a celiac patient must not respond well to a gluten-free diet and the GCED diet.
Then her doctor must also determine with certainty that she doesn’t suffer from Crohn’s disease, fructose intolerance, lactose intolerance, microscopic colitis, pancreatic insufficiency, small intestine bacterial overgrowth (SIBO), or ulcerative colitis.
And finally the lining of the small intestine will have an abnormal T-cell population.
Type 2 refractory celiac disease is very rare and very serious. As I stated in my celiac disease symptoms lesson, patients with RCD2 have a high chance of developing Enteropathy-associated T-cell Lymphoma (EATL), a serious malignant cancer.
This is why you must be absolutely certain gluten isn’t sneaking into your life somehow and you must work through this diagnosis with an experienced medical professional.
So as not to scare you, please remember that a vast majority of patients with non-responsive celiac disease respond well to the strict GCED diet and ultimately aren’t diagnosed as having type 1 or type 2 RCD.
The Gluten Contamination Elimination Diet
The GCED diet is a very strict diet designed to avoid any possible trace of gluten cross-contamination. Here are the basics of this diet, but I encourage you to view my lesson dedicated to this diet.
The basics sound simple: Avoid all grains accept white and brown rice, avoid dairy, and avoid anything with an ingredient list. That means no packaged, canned, frozen or dried foods whatsoever. It also means no deli slices or ham (including bacon), which are often processed in some manner.
To read more details on this strict but effective diet, including a nice clear table of what to avoid and what you can still have, visit my lesson:
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Immunosuppressive Medications (Steroids)
For patients who don’t respond to the GCED diet and are ultimately diagnosed with refractory celiac disease, immunosuppressive medications may eventually be prescribed and symptoms will be monitored.
Prior to trying immunosuppressive medications, doctors try a combination of pancreatic supplements and probiotics. After testing and tweaking different combinations of pancreatic supplements and probiotics, if the patient still doesn’t show signs of healing, the doctor will resort to strong medications.
RCD1 patients usually respond well to the following medications:
Immuno-suppressive drugs (cyclosporines and azathioprine (Imuran))
While these medications may suppress the antibody activity inflicting damage to your small intestine, they also suppress many natural and healthy immune system functions. So while taking these medications, you will need to maintain clear and regular communication with your physician.
This is also why you really, really want to make sure you’ve eliminated gluten from your diet and at least tried the GCED diet before being diagnosed and treated for refractory celiac disease.
Recent research suggests cladribine therapy (2-chlorodeoxyadenosine or 2-CdA) may be effective for both RCD1 and RCD2 patients.
The Potential Consequences of Persistent Villous Atrophy
Conclusion and Review
Before you accept a refractory celiac disease diagnosis, make sure your doctor is aware of the most recent research involving gluten cross-contamination and the GCED diet.
If you still aren’t well, you want to make sure your doctor also thoroughly assesses you for the digestive conditions that can influence your health and test results. Again, these conditions include Crohn’s disease, fructose intolerance, intestinal lymphoma, lactose intolerance, microscopic colitis, pancreatic insufficiency, small bowel bacterial overgrowth, or ulcerative colitis.
Refractory celiac disease is now considered even more rare than it used to be, so for a majority of people reading this lesson, there is great hope in implementing the Gluten Contamination Elimination Diet.
Take The Quiz!
How Well Do You Understand Effective Refractory Celiac Disease Treatment?
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