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  • Jefferson Adams
    Jefferson Adams
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    Gluten-Free Diet and Steroids Effective for Most Cases of Collagenous Sprue

    Caption: Steroids and a GFD to Treat Collagenous Sprue

    Celiac.com 05/04/2010 - A team of clinicians recently set out to assess the effectiveness of treating collagenous sprue with a combination of gluten-free diet and steroids.

    The team was made up of Alberto Rubio-Tapia, Nicholas J. Talley, Suryakanth R. Gurudu, Tsung-Teh Wu, and Joseph A. Murray. They are affiliated variously with the Division of Gastroenterology and Hepatology of the Mayo Clinics in Scottsdale, Arizona, Jacksonville, Florida, and Rochester, Minnesota, and the Division of Anatomic Pathology in Rochester Mayo Clinic.

    Deposits of subepithelial collagen that form a distinctive band in the small bowel are one of the clinical hallmarks of collagenous sprue.

    For the study, the team evaluated clinical characteristics, treatments, and outcomes of patients with collagenous sprue. The team looked at medical records for thirty patients with collagenous sprue from the Mayo Clinics from Scottsdale, Jacksonville, and Rochester, for the periods covering 1993 and 2009.

    21 of the patients were female (70%), ranging in age from 53–91 years. The majority of patients suffered from severe diarrhea and weight loss.

    However, collagenous spore is commonly associated with collagen deposits or chronic inflammation in other parts of the gastrointestinal tract, as well as other immune-mediated disorders.

    16 patients (53%) were hospitalized to treat dehydration, while 21 patients (70%) suffered from associated immune-mediated diseases, the most common of which was celiac disease. Other common associated diseases included microscopic colitis, hypothyroidism, and autoimmune enteropathy.

    Subjects showed subepithelial layers of collagen deposits in the small bowel ranging from 20 –56.5μm, and averaging 29 μm thickness. Eight patients showed subepithelial collagen deposits in the colon or stomach.

    24 patients (80%) showed a positive clinical response to treatment with a combination of a gluten-free diet and immunosuppressive drugs. Nine patients showed confirmed histologic improvement, while five patients experienced complete remission. Of two patients who died, one succumbed to complications from collagenous sprue, while one died of another illness.

    Most patients with collagenous sprue show a positive clinical response to a combination of gluten-free diet and steroids.

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  • About Me

    Jefferson Adams earned his B.A. and M.F.A. at Arizona State University, and has authored more than 2,000 articles on celiac disease. His coursework includes studies in biology, anatomy, medicine, and science. He previously served as Health News Examiner for Examiner.com, and provided health and medical content for Sharecare.com. Jefferson has spoken about celiac disease to the media, including an appearance on the KQED radio show Forum, and is the editor of the book "Cereal Killers" by Scott Adams and Ron Hoggan, Ed.D.

  • Related Articles

    Michelle Melin-Rogovin
    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:
    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.

    Jefferson Adams
    Celiac.com 09/12/2012 - A team of researchers recently evaluated tioguanine as a treatment for refractory celiac disease type I. The very small study indicates that tioguanine, a thiopurine derivative, offers easy, efficient treatment for refractory celiac disease, compared with current treatment regimens.
    The research team included G. J. Tack; D. P. van Asseldonk; R. L. J. van Wanrooij; A. A. van Bodegraven; and C. J. Mulder.
    Refractory celiac disease type I is a form of celiac disease in which patients show resistance to a gluten-free diet, and suffer persistent or recurring intestinal villous atrophy, along with symptoms of malabsorption.
    Currently, the most common treatment for refractory celiac disease type I relies on corticosteroids, though azathioprine is also sometimes used.
    However, this small recent study shows that tioguanine might be better tolerated and more effective, in part because of its simpler metabolism towards bio-activation.
    For their study, the research team set out to assess how well patients with refractory celiac disease type I tolerate tioguanine, and how effective it is in relieving symptoms.
    The team studied a group of twelve patients with refractory celiac disease type I, who were treated with tioguanine between June 2001 and November 2010, including a follow-up period of at least 1 year.
    The team assessed and recorded adverse events, laboratory values, 6-thioguanine nucleotide concentrations and rates of both clinical and histological response at baseline and during follow-up.
    They noted that the average tioguanine treatment lasted 14 months. Ten patients tolerated long-term tioguanine treatment, while two patients discontinued treatment due to adverse reactions.
    The team found no nodular regenerative hyperplasia of the liver. Follow-up showed clinical and histological response in 83% and 78% of patients, respectively. Overall, patients decreased corticosteroid reliance by 50%.
    Because of its higher histological and similar clinical response rates compared with current treatments, tioguanine seems to be a good drug for treating refractory celiac disease type I.
    Source:
    Alimentary Pharmacology & Therapeutics. 2012;36(3):274-281.

    Jefferson Adams
    Persistently High Antibodies Reveal Non-responsive Celiac Disease
    Celiac.com 04/07/2014 - Histologically non-responsive celiac disease (NRCD) is a potentially serious condition found in celiac disease patients who suffer persistent villous atrophy despite following a gluten-free diet (GFD).
    Currently, the only way to monitor patient progress rely on invasive and costly serial duodenal biopsies. Looking for better options, a team of researchers recently set out to identify antibody biomarkers for celiac disease patients that do not respond to traditional therapy.
    The research team included B. N. Spatola, K. Kaukinen, P. Collin, M. Mäki, M. F. Kagnoff, and P. S. Daugherty. They are affiliated with the Department of Chemical Engineering, University of California, Santa Barbara in California, the Department of Gastroenterology and Alimentary Tract Surgery and the Center for Child Health Research at the University of Tampere and Tampere University Hospital in Tampere, Finland, with the Department of Medicine at Seinäjoki Central Hospital in Seinäjoki, Finland, and with the Laboratory of Mucosal Immunology in the Departments of Medicine and Pediatrics at the University of California San Diego in La Jolla, California.
    Using flow cytometry to screen bacterial display peptide libraries, the team was able to identify the epitopes specifically recognized by antibodies from patients with NRCD, but not by antibodies from responsive celiac disease patients.
    By comparing ELISA results for sera from 15 NRCD patients and 45 patients with responsive celiac disease, all on a strict GFD for at least 1 year, the team confirmed that deamidated gliadin was the antigen mimicked by library peptides.
    They identified the dominant consensus epitope sequence by unbiased library screening QPxx(A/P)FP(E/D). The epitope sequence was highly similar to reported deamidated gliadin peptide (dGP) B-cell epitopes.
    They also found that anti-dGP IgG measurement by ELISA discriminated between NRCD and responsive celiac disease patients with 87% sensitivity and 89% specificity.
    Most importantly, they found that dGP antibody levels correlated with the severity of mucosal damage, meaning that IgG dGP levels may be useful in monitoring small intestinal mucosal recovery on a GFD in NCRD patients.
    The team found that celiac patients with NRCD can be spotted by their increased levels of anti-dGP IgG antibodies even when the patients are following strict gluten-free diets
    Lastly, they feel that anti-dGP IgG assays may be useful for monitoring mucosal damage and histological improvement in celiac disease patients on a strict GFD.
    Source:
    Aliment Pharmacol Ther. 2014;39(4):407-417.

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    Report on Gluten Free Foods & Beverages Market (2019) gives complete outlook of ... non-celiac patients with more number of new product introductions in ... Awareness of Celiac Disease and Gluten – Intolerance Consumers 3.1.2 ... View the full article
    Okay, thanks. Re: MTHFR - I don't really know yet... I only started reading about it yesterday and it is pretty overwhelming. But it does seem to be common advice that if you have a close relative with it you should be tested, and I guess having 2 copies of the "C" variant, as my sister has, is the "worst" variety of it.  It came to light for her when she was going through infertility and miscarriages.  They discovered that her homocysteine was high, which led to the MTHFR testing. So that is one thing I know I would then want to proceed to do, if I do have it - get my homocysteine tested. My dad died of early-onset Alzheimer's, and apparently there is a link between high homocysteine as well as the MTHFR mutation and Alzheimer's. It also seems like it would be worth knowing if I have it since it could be the cause of my lower levels of B12. And I guess maybe I would need to start taking methyl-folate? I mean, to answer your question, I am not entirely sure what I will do if I do have it.   Probably read a lot more about it... and take supplements like methyl-folate if I really think I need to.  Check my homocysteine & control that if I need to, hopefully to lower my risk of Alzeheimer's.  It seems like a frustrating area because there appear to be limited official medical websites that really even talk much about it (so far).  I have found one article on the NIH that focuses on the link with high homocysteine. I already eat a very healthy diet.  Whole grains, lots of fruit & veg, mostly organic.  I am a vegetarian except for very rare seafood. I avoid processed food and, above all, foods with added sugar...  To me, sugar is by far the worst culprit in the SAD.  I think RA has been ruled out by my 2 negative Rheumatoid Factor tests (one done several years ago, one just this year at my physical).  Also, the way this started in my elbows, and was really only there for years, is just... weird... and definitely doesn't really fit with arthritis.  And there is no swelling to speak of, just mild pain - sometimes aching, sometimes burning, sometimes sharp...  It may or may not fit with any systemic diagnosis versus a mechanical one, but nowadays I do also have pain in my hands, feet, and knees.  So then I think, well maybe it is/was something systemic, but it was worse in my elbows for some mechanical reason but now has progressed elsewhere.  I thought Crohn's was just digestive?  (Of course, many people think that of celiac.)  So I haven't really investigated that one much. My ANA was retested and is back down to "negative," so I think that pretty much rules out lupus.  I believe fibromyalgia is still on the table. Anyhow....  Your point is nonetheless taken.  I do want to rule out celiac and go from there.  At this point I'd sure love to find out it is something I could control through my diet!
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