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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: Presence of persistently damaged villi in the small intestine that are not repaired after the gluten free diet has been successfully initiated and/or maintained An increased presence of intraepithelial lymphocytes (IEL) in the small bowel Severe malabsorption 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. 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 06/21/2013 - Caroline, a third-grader at St. Pius school in Chicago her mother, Cassandra, both have celiac disease. After being formally diagnosed at the Celiac Disease Center at the University of Chicago, they each received care package that contained "…lots of gluten free foods, as well as tons of literature about eating gluten free,” said Cassandra. “Caroline’s care package had a stuffed animal in it," she added. The university's care package program is funded solely by donations, and this knowledge, along with Caroline's appreciation, led to a desire to support the Celiac Disease Center. Cassandra credits Caroline with a plan to make and sell pony tail holders with ribbons. Caroline made the holders herself, and sold them for $4.00 each, collecting over one hundred dollars in the process. Caroline specifically "wanted the money to be used to send another little girl or boy a care package and stuffed animal,” said Cassandra. Caroline proudly announced her efforts and presented the money at her at her annual appointment with Dr. Stefano Guandalini, founder of the University of Chicago Celiac Disease Center. Dr. Guandalini was "so appreciative and proud of her efforts,” beamed Cassandra. “His kind words to her made our day, and Caroline left feeling great about giving back!” Principal Daniel Flaherty called Caroline a "…great example for all of us here at St. Pius X Parish School.” Source: http://www.chicagotribune.com/news/local/suburbs/lombard_villa_park/community/chi-ugc-article-st-pius-x-third-grader-inspired-to-give-2013-04-26,0,5389721.story

For the first time, researchers at the University of Chicago Celiac Disease Center will use mouse model research to explore root causes of celiac disease, test new therapies, and explore new targets for treatment. Celiac disease is the most common genetic autoimmune disease in the world. Celiac disease affects approximately three million Americans, but only three out of every one hundred people with celiac disease have been diagnosed. At least ninety-seven percent of people with celiac disease undiagnosed. Untreated celiac disease can lead to osteoporosis, infertility, neurological conditions, and cancer. Moreover, people with celiac disease have a substantially higher risk of developing other autoimmune diseases, especially Type-1 diabetes. Bana Jabri, M.D., Ph.D., Associate Professor, University of Chicago Medical Center, and a leading celiac disease researcher, will create the new mouse model with the goal of identifying new remedies and preventive treatments targeted at children of families with a history of celiac disease. The studies will also investigate events that contribute to the development of Type-1 diabetes. “There is a critical need to provide the proper resources to those who suffer from celiac disease,” said Stefano Guandalini, M.D., professor of pediatrics at the University of Chicago Medical Center, founder and medical director of the Celiac Disease Center. “This commitment from University of Chicago Celiac Advisory Board reaffirms the Celiac Disease Center’s mission to bring cutting edge research, education and encouragement to those affected by the disease”. Dr. Jabri believes that mouse models are central to understanding the underlying causes of celiac disease, its connection to other autoimmune diseases. The University of Chicago Celiac Disease Center is a 501-c3 non-profit organization, completely funded by donor contributions, and committed to improving the care, diagnosis and awareness of celiac disease. The University of Chicago Celiac Disease Center also provides necessary infrastructure and support for cutting-edge celiac research, including investigations into structure of gluten peptides and the mechanisms by which gluten modifies self molecules. Mouse model studies show promise in helping researchers to better and more quickly unlock the secrets of celiac disease. For more information please visit: www.celiacdisease.net