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Refractory Sprue by Michelle Melin-Rogovin, University of Chicago Celiac Disease Program

This article originally appeared in the Spring 2003 edition of Celiac.com's Scott-Free Newsletter.

Refractory sprue. The specter of this condition is enough to cause fear in the hearts of many people living with celiac disease, yet this fear is based more on myth and misunderstanding than on medical science. For those who are concerned about their risk for developing refractory sprue, there is much that can be done. For those who have developed the condition, there are treatment options and new hope on the horizon. To begin, however, we must substitute fear with knowledge.

What is refractory sprue?
This question has been the subject of great scientific inquiry, and there are differing opinions on the relationship between celiac disease and refractory sprue. However, there are several general characteristics of refractory sprue that researchers seem to agree on:

Researchers think of celiac disease as the beginning of a spectrum of conditions that could, for a small percentage of patients, end up at the other end to be enteropathy associated T-Cell Lymphoma. Most people with celiac disease will respond to the gluten free diet and never move to the next stage in this spectrum. But for those that do, they will experience changes in their immune system and in the cells lining their intestine that could lead to cancer.

The spectrum would start with celiac disease, and the next step would be the non-responsiveness of the immune system to the gluten-free diet, in other words, refractory sprue. Then in some cases, a condition called ulcerative jejunitis develops, and finally, the damaged lining of the intestine produces cancer cells that mimic the mutations of the abnormal immune system cells.

How many people with celiac disease are affected by refractory sprue?
First, there are no reported cases in the medical literature of celiac sprue in people under 20 years of age. Second, the number of celiacs affected by refractory sprue, while not known, appears to be very small. We know this because the current estimates for small bowel cancers in people affected by celiac disease, as reported at the 10th International Conference on Celiac Disease is less than 2.5%. Refractory sprue can result in small bowel cancers, but not in all cases.

It is interesting to note that in a recent study of patients with "unresponsive" celiac disease, Dr. Joseph Murray and his colleagues found that of 49 patients evaluated, only nine actually had refractory sprue—25 were found to have gluten contamination in their diets. The most common symptoms presented by the patients who truly had refractory sprue were weight loss, steatorrhea and diarrhea, in that order.


What makes refractory sprue different than celiac sprue?
Again, there are several medical points of view on this, but all researchers would agree that one marker indicates the presence of refractory sprue, and it is not found in celiac disease.

Abnormal Intraepithelial Lymphocytes (Immune Cells)
The intraepithelial lymphocytes found in celiac disease have a normal-looking appearance under the microscope and they behave like normal celiac immune cells (they respond to gluten when they shouldnt). These lymphocytes have the ability to communicate with other cells using different types of messages on their cell surfaces. When diagnosing celiac disease, pathologists look for an increased number of IELs as an indication of celiac disease.

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In refractory sprue, however, there is a different kind of IEL that is found in great numbers. This immune cell does not look normal, and it ignores the presence or absence of gluten. This type of cell does not have the ability to communicate normally with other cells as it would be expected to do. However, it does have the ability to communicate with cancer cells, contributing to their development. It is not clear what causes this type of IEL to develop or mutate, contributing to refractory sprue.

It is possible to have refractory sprue without having these abnormal lymphocytes; in this case, treatment with steroids often results in response to the gluten free diet and a reversal of the condition.

French researchers have developed a test to determine whether a biopsy specimen reflects a normal course of celiac disease with a slow response to the diet, or the need for further testing because refractory sprue may be present. In paraffin wax, a specimen can be stained to determine whether or not the immune cells express CD8, a protein often found on intraepithelial lymphocytes in celiac disease. If CD8 is positive, the individual has celiac and is responding very slowly to the diet. If the sample is CD8 negative, refractory sprue could be the reason.

How is refractory sprue diagnosed and treated?
It must be established through a thorough diet history and antibody testing that the individual is adhering to a strict gluten-free diet. Then, all other gastrointestinal diseases have to be ruled out before a diagnosis of refractory sprue is made. Conditions to be ruled out include pancreatic insufficiency, lactose malabsorption, parasite infestation, intolerance to other food proteins, coexisting inflammatory bowel disease, and autoimmune enteropathy, among others.

Diagnosis should include a test called an enteroscopy, which is a procedure that explores more of the small intestine, and often finds ulcerative jejunitis, a marker of damage in refractory sprue. In addition, because the abnormal IELs can proliferate throughout the gut, a colonoscopy is recommended to determine if lymphocytic colitis is present.

Treatment options include the elemental diet (also used in Crohns Disease), total parenteral nutrition (tube feedings), steroids, immunosuppressive therapies such as Cyclosporine, Infliximab, and in some cases, chemotherapy. Treatment options depend on the extent of refractory sprue found on biopsy and the nature of the clinical symptoms involved.

How can I reduce the chances of developing refractory sprue?
Researchers agree that most cases of refractory sprue develop in people who were diagnosed very late in life or who didnt follow the diet completely. Note that it doesn't matter how much gluten was consumed in these patients, they still developed refractory sprue. So the best protection against developing refractory sprue is to follow the diet. Be honest with yourself, especially if you cheat a little. What are you eating? Are you sure there isnt a great gluten-free alternative out there? Hey, there's even beer nowadays, so don't dismiss the suggestion of great gluten-free brownies, cakes, pies, pasta, crackers, cookies, or whatever else you are craving.

Deal with your feelings too. Its easy to get angry about how life is much harder for people with celiac disease—how everything related to food requires too much planning, preparation, and explanation. These feelings are perfectly justified, but they do not justify cheating on your diet. There are great "quick fix" cookbooks out there, even convenience meals that are gluten free. Do whatever it takes to stay healthy, and gluten-free for life.

Don't forget regular visits to your gastroenterologist or internist. Follow-up care for people with celiac disease is incredibly important, even if the medical community hasn't recognized it yet. Regular antibody testing to monitor compliance with the diet is an extra level of protection that every celiac needs. A simple anti-gliadin antibody test (IGG and IGA), six months post diagnosis, a year post-diagnosis and then every year after that for the first three years is key. In fact, the most serious celiac disease complications tend to occur in the first three years after diagnosis. Veteran celiacs should have their antibody levels checked every couple of years.

While refractory sprue remains a potential complication for any adult with celiac disease, a majority of adult celiacs in this country will not have to face this difficult condition. For those diagnosed, treatment options continue to improve and the disease is becoming easier to manage. Researchers continue to study refractory sprue in order to better understand how the condition behaves and to develop new treatments. For now, the best defense against refractory sprue is a good offense—living a completely gluten-free life.

Celiac.com welcomes your comments below (registration is NOT required).





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12 Responses:

 
dennis
Rating: ratingfullratingfullratingfullratingemptyratingempty Unrated
said this on
01 Nov 2007 2:33:59 PM PDT
How does one cheat on this diet? If I get cross-contamination I'm horribly sick for three days. I almost died before doctors figured it out. I actually stumbled on to it when talking to some friends who were nurses.

 
an unknown user
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said this on
06 Feb 2008 6:52:20 AM PDT
This is an excellent article that is easy to understand. It would be nice if more doctors would understand this.

 
Celiac Scott
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said this on
02 Mar 2008 10:03:21 AM PDT
A terrific article. After a two month strict diet I redeveloped all the symptoms again despite a strict diet. My Dr. jumped to the conclusion that I had refractory sprue and wanted a section of my intestine removed. I write this from the surgical recovery ward after surgery. I was opened up and re-closed after inspection showed nothing more than an infection. I needed more testing as described in this article prior to surgery. Oh well, I have a great scar and a great lesson learned. GET A SECOND OPINION!

 
Mary Rodgers
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said this on
22 Oct 2011 11:13:27 PM PDT
So much information - thank you. Your articles are easy to understand. Don't go away -- we need you !!!

 
Ilir
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said this on
21 May 2012 12:10:41 PM PDT
Thank you very much for putting everything in the context and clearly explaining such a complex issue.

 
Virginia Mei
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said this on
25 May 2012 3:47:47 PM PDT
I wish my doctor explained it like you do. I was just diagnosed with refractory celiac sprue and I am very scared. Unfortunately he has very little time to answer my questions which makes me glad you are there explaining this awful disease.

 
G kirma
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said this on
25 Jun 2012 3:23:49 PM PDT
Virginia, I'm sorry to hear your doctor doesn't spend more time with you. I was diagnosed with celiac disease in 1992 and have been 100% compliant with a gluten-free diet. I'm also a lifelong insulin-dependent type 1 diabetic and vegetarian by choice. Since May 11 I've had horrendous diarrhea and my doctor can't figure out what is wrong. I'd love to hear from you and perhaps we can help each other. Thanks for considering it.

 
Doug Jackson
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said this on
02 Nov 2012 9:44:16 AM PDT
Thank you for helping to make this life-changing disease a little more understood. I was first diagnosed with celiac disease 20 years ago and as I weaned off the steroids, my symptoms came back. I have been on the steroids ever since, and many doctors have failed to figure out what was wrong with me. I was recently sent to The Cleveland Clinic for evaluation and was subsequently diagnosed with refractory sprue. I had a central IV line installed in my chest for TPN feeding. I will need to be tube fed for at least a few months due to severe malnutrition over a very long time. It is possible the tube feeding will be indefinite. I sincerely hope that there is a cure on the horizon. One would think that if doctors can give a person an actual bionic leg (which I just read about) then they could develop an enzyme that would help digest and absorb gluten. Something.

 
kim
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said this on
20 May 2014 11:18:54 AM PDT
I was just diagnosed with refractory sprue after a second colonoscopy and endoscopy. I have at least 6 to eight 8 loose stools every day. I didn't think anything of it really until my stomach started to hurt every day. Is diarrhea normal for a person on a strict gluten free diet?

 
Theresa
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said this on
14 Aug 2014 3:44:59 AM PDT
This is a great article. I have been diagnosed with celiac for 11 years now, eating a strict gluten free diet, other than accidental contamination, which we have further researched and cleared up. It took nearly 8 yrs for my villi to show any change. I am a mystery to my doctors. I was diagnosed with lymphocytic colitis a few years ago. I was on high dose steroids, no effect, other than the negatives of high dose steroids. I still suffer from diarrhea a few times a day and during the night. It is watery now, it's getting worse in some ways, and I have removed more food groups! I will have to get some further help when we have the funds. I think I understood the relationship between refractory and LC - not sure. I had one specialist at the Lahey Clinic tell me it was probably refractory sprue, but didn't suggest any followup. I am confused. I know there is hope out there!!! Thanks for the details on what to have tested!!!

 
Yuri
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said this on
18 Apr 2015 8:44:16 AM PDT
An informative article with excellent information but if you have refractory sprue you cannot have packaged goodies that are labeled gluten free. Gluten free does not mean anything to manufactures and 20ppm or less gluten in a product does NOT make it gluten free! It just makes refractory people sick. If you have celiac sprue just cook everything at home if you have refractory sprue do the same.

 
virginia
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said this on
17 Aug 2015 5:39:30 PM PDT
Has anyone developed this after chemotherapy for B cell lymphoma? I had controlled celiac for 22 years. after second infusion I have had diarrhea for over a year. I am very wasted and no medication has really helped. Wonder where is the best place for diagnosis of this disorder?




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Hi! My daughter is 19 was diagnosed at age 16. It took about 12-18 month s for her to fully heal from the damage and feel "normal" again. Also because of the damage done she had reactions to dairy, so you may want to try no or minimum dairy until youre fully healed. Just a suggestion. Hope you start feeling well soon!

Hi yall! New to this blog, but really glad it exists because I have lots of questions. First off, I'm Allie! I'm 17 and newly diagnosed Celiac after about 3 years of searching for answers. I initially went gluten-free on the recommendation of a friend, I felt better in about a month and then my pediatric gastroenterologist had me do the gluten challenge, and my symptoms were the worst they have ever been, and ones I barely noticed before became very present. I did the biopsy and was diagnosed, it's been about 2 weeks and my symptoms are still pretty bad, although my diet has no known sources of gluten or cross contamination. Wondering if anyone has any input on healing post gluten challenge, any tips or how long it took for you would be quite helpful! Thanks

Might want to look into a keto diet, I have UC on top of celiacs and keto is working great Yeah I have major nerve and brain issues with gluten, gluten ataxia with nerve issues and brain issues. Seems to cause my body to attack my brain and nerve system. My brain stumbles fogs, and starts looping, the confusion causes me to become really irritable, I call it going Mr Hyde. Like my mind will start looping constantly on thoughts and not move driving me literally mad, or it used to. Now days it is primary the numbness anger but the gut issues and sometimes random motor loss limit me motionless to the floor now days for the duration of the major anger effects. Used to be a lot more mental then painful gut. I did a mental trauma post on it on while back where I came out about all my mental issues with gluten.

^^^^^^ good info, tips and tricks^^^^^^^^^ yes, crumbs will make you sick. also, breathing flour/pancake mix, etc that is in the air because eventually, you're going to swallow some.

Hello I was diagnosed Dec 15 of last year and went totally gluten-free the next day. I actually got worse before I got better - it's a steep learning curve - but now, 4 1/2 months later I'm finally seeing improvement. Hang in there.