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Celiac.com 01/09/2025 - Celiac disease is a chronic autoimmune condition triggered by consuming gluten, a protein found in wheat, barley, and rye. For most individuals with this condition, following a strict gluten-free diet is the key to managing symptoms and promoting intestinal healing. However, not all patients experience relief, even after adhering to this diet for six to twelve months. This condition, termed non-responsive celiac disease, has now been the subject of a comprehensive study analyzing its prevalence and causes. What Is Non-Responsive Celiac Disease? Non-responsive celiac disease refers to the persistence of symptoms such as diarrhea, abdominal pain, and malnutrition despite maintaining a gluten-free diet for an extended period. This phenomenon can arise either due to ongoing gluten consumption—knowingly or unknowingly—or because of other underlying medical conditions. According to the study, approximately 20 percent of individuals with celiac disease do not respond to a gluten-free diet as expected. This alarming proportion highlights the complexity of managing celiac disease and the need for further understanding of why some patients continue to suffer. The Most Common Cause: Hidden Gluten Exposure For one-third of patients with non-responsive celiac disease, the main culprit is inadvertent gluten exposure. Gluten is ubiquitous, often hiding in processed foods, sauces, and even medications. Even trace amounts can provoke an immune reaction in sensitive individuals. Many patients are unaware that they may still be consuming gluten, either due to poor food labeling or a lack of education about gluten-containing products. This underscores the need for better awareness, improved labeling regulations, and ongoing dietary counseling for individuals newly diagnosed with celiac disease. Other Causes of Persistent Symptoms Functional Gastrointestinal Disorders The study found that 16 percent of cases of non-responsive celiac disease were linked to functional gastrointestinal conditions, such as irritable bowel syndrome. These disorders, which are not caused by structural abnormalities or ongoing gluten exposure, often mimic celiac disease symptoms, making diagnosis and treatment challenging. Refractory Celiac Disease In rare but serious cases, symptoms persist due to a condition called refractory celiac disease. This occurs when the immune system continues to attack the small intestine despite strict adherence to a gluten-free diet. Refractory celiac disease is further divided into two types: Type I: Generally responds well to treatment and follows a milder course. Type II: Associated with a higher risk of progression to lymphoma, a form of cancer. Refractory celiac disease, while less common, represents a significant concern because of its potential for severe complications. Misdiagnosis or Other Conditions In some cases, a misdiagnosis of celiac disease could explain ongoing symptoms. Alternatively, other conditions such as small intestinal bacterial overgrowth, lactose intolerance, or inflammatory bowel disease may be the true cause of persistent issues. A thorough medical evaluation is crucial for ruling out these possibilities. Implications for Healthcare The findings of this study highlight several critical areas for improving care for individuals with celiac disease: Enhanced Dietary Education Patients need comprehensive guidance on identifying and avoiding hidden gluten sources. This includes recognizing potential cross-contamination in kitchens, understanding food labels, and staying vigilant about gluten-free certification. Better Food Labeling Standards Gluten labeling varies widely across countries, with some regions lacking clear regulations. Standardized global practices could help reduce inadvertent gluten exposure and improve quality of life for celiac patients. Targeted Medical Interventions For those with non-responsive celiac disease, a personalized approach is essential. This may include testing for other conditions, functional disorders, or refractory celiac disease. Additionally, new therapies targeting persistent symptoms are being developed, offering hope for those who do not respond to dietary changes alone. Why This Study Matters For individuals living with celiac disease, non-responsive cases can be particularly distressing. The persistence of symptoms can lead to ongoing health issues such as malnutrition, anemia, and decreased bone density, not to mention the emotional toll of chronic illness. This study emphasizes the importance of addressing all potential causes of persistent symptoms and tailoring care to individual needs. By identifying the main drivers of non-responsive celiac disease—such as hidden gluten and functional gastrointestinal disorders—it provides a roadmap for improving diagnosis, treatment, and overall patient outcomes. Ultimately, these findings remind us that while a gluten-free diet remains the cornerstone of celiac disease management, it is not always a cure-all. Continued research, enhanced education, and more effective treatments are essential to supporting the one in five patients who do not find relief from dietary changes alone. Read more at: onlinelibrary.wiley.com
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Celiac.com 01/06/2023 - Non-responsive celiac disease (NRCD) affects up to 15% of children with celiac disease. A Gluten Contamination Elimination Diet (GCED) is a more stringent diet consisting of fresh, whole, and unprocessed naturally gluten-free foods. A team of researchers recently set out to assess their approach to identifying and treating NRCD with budesonide and the Gluten Contamination Elimination Diet (GCED). Their results were encouraging. Here's what they found. The research team included Awab Ali Ibrahim, Victoria Kenyon, Alessio Fasano, and Maureen M Leonard. They are variously affiliated withthe Division of Pediatric Gastroenterology and Nutrition, MassGeneral Hospital for Children, Harvard Medical School, Boston, MA; the Department of Pediatrics, Harvard Medical School, Harvard University, Boston, MA; the Center for Celiac Research and Treatment, MassGeneral Hospital for Children, Harvard Medical School, Boston, MA; the Mucosal Immunology and Biology Research Center, MassGeneral Hospital for Children, Boston, MA; the Celiac Research Program, Harvard Medical School, Boston, MA. NRCD Defined Non-responsive celiac disease is defined as patients having persistent symptoms and enteropathy, with at least Marsh 3 histology, after following a gluten-free diet for at least 12 months. Researchers think that NRCD affects up to 15% of children with celiac disease, but there is limited data, and no research to date, describing treatment of children with this NRCD. Retrospective, Single Center Analysis The team performed a retrospective, single center analysis over a 5-year period of patients with celiac disease 18 years of age and under, who received treatment for persistent symptoms and enteropathy despite following a gluten-free diet. NRCD Patients Respond to GCED and Budesonide The team found a total of 22 patients with NRCD. Of the thirteen patients treated with the GCED for 3 months, nearly half experienced both histological and symptomatic resolution of celiac disease. Of the nine patients were treated with budesonide (6-9 mg), nearly ninety percent experienced both symptomatic and histologic resolution after treatment averaging three months. Further, more than two-thirds of patients who responded to the GCED, and 100% of patients who responded to budesonide, experienced remission of at least 6 months following treatment transition back to a gluten-free diet. Treatment of NRCD with the GCED and budesonide can provide benefit most NRCD patients. Most patients with NRCD can return to a standard gluten-free diet after about three months of treatment. This is some of the most promising treatment information we've seen with regard to NRCD. The article shows that many celiac patients not responding to a gluten-free diet can respond to a more stringent approach. The high response rate to this treatment offers exciting news for patients with NRCD and their physicians. Stay tuned for more on this and related stories. Read more at J Pediatr Gastroenterol Nutr. 2022 Nov 1;75(5):616-622.
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Celiac.com 12/21/2018 - For most celiac patients, treatment with the gluten-free diet marks the turning point for their health. It can take a few months for the villi of the small intestine to heal, but eventually the villi are able to absorb the nutrients in their food and the symptoms of celiac disease are alleviated. Unfortunately, there are some celiacs who don’t respond to the gluten-free diet. This is the only current treatment for the disease, resulting in a condition known as refractory celiac disease or nonresponsive celiac disease (NRCD). Although celiac experts have stated that actual refractory celiac disease, wherein damage to the small intestine is irreversible, is rare, a preliminary study reported by Med Page Today suggests that the condition is more common than the medical community once thought. Fortunately, the study also showed that refractory celiac disease patients did respond favorably to medical treatment. Celiac disease, an autoimmune disease caused by gluten, a protein found in wheat, barley, and rye, affects three million Americans, or about 1% of the U.S. population. Patients with refractory celiac disease experience abdominal pain, severe malabsorption of nutrients, and intestinal damage. A single-center preliminary study suggests that “more patients with celiac disease may stop responding to their gluten-free diets,” as reported by Med Page Today. The researchers studied non-responsive celiac patients treated at the University of Virginia Medical Center over the past decade. According to Med Page Today, “Overall, patients were diagnosed with refractive disease a mean of 4.7 years following their initial diagnosis of celiac disease.” Furthermore, diagnoses of refractory celiac seemed to occur more recently, mostly within the last five years and almost half of them within the last six months. The researchers, including Christopher Hammerle, MD, and Sheila Crowe, MD, of the University of Virginia in Charlottesville, found that the refractory celiac disease patients did respond to treatment with thiopurines. “These agents are my treatment-of-choice for refractory celiac disease to avoid long-term steroids,” Hammerle told MedPage Today. According to Shailaja Jamma, MD, and Daniel Leffler, MD, MS, in Real Life with Celiac Disease, there could be many explanations for a failure to respond favorably to the gluten-free diet. In their chapter on NRCD, they write, “you would need to be on a GFD for at least 6 months without significant improvement before we would decide that you were not responding and look for other reasons.” This is due to the fact that recovery times vary from person to person, and as long as patient seems to be improving continually over time, no matter the speed, non-responsive celiac disease is usually an unnecessary label. Jamma and Leffler found that the most common causes—designated “very common’—are gluten exposure and Irritable Bowel Syndrome (IBS). The next most common causes of NRCD, labeled as “somewhat common,” are lactose intolerance or fructose malabsorption, microscopic colitis, and small intestinal bacterial overgrowth. “Rare” causes include actual refractory celiac disease, which can be confirmed with a biopsy of the small intestine, an eating disorder, inflammatory bowel disease, which can also be confirmed with a biopsy as well as imaging studies of the small or large intestine, pancreatic exocrine insufficiency, and motility disturbances, that is, when food moves too quickly or too slowly through the intestine. Finally, food allergy and cancer are “very rare” causes of NCRD. According to the Mayo Clinic, as reported by Celiac.com, “gluten contamination is the leading reason for non-responsive celiac disease,” and estimates that 18% of non-responsive celiac disease cases are due to actual refractory celiac disease. The Mayo Clinic researchers recommend that before making a refractory celiac disease diagnosis, additional diseases as well as gluten contamination should be ruled out as causes. According to Jamma and Leffler, “The first step is often to get confirmation that you do indeed have celiac disease,” since “celiac disease can be mistakenly diagnosed when the true problem is something else.” Med Page Today points out that most of the patients with refractory celiac disease responded favorably to a thiopurine medication rather than the conventional method of treatment for the condition, steroids. This form of treatment doesn’t carry with it the risk of steroid dependence. If you have some concerns regarding your response to the gluten-free diet, it’s recommended that you talk with your doctor about a non-responsive celiac disease evaluation. An evaluation of your diet may very well confirm that you are still ingesting gluten, but if this isn’t the case, other causes can be explored by your doctor. Thiopurine seems promising as a treatment option for those who do, in fact, have actual refractory disease. Resources: 1. About.com: Refractory (Unresponsive) Celiac Disease 2. Celiac.com: Causes of Non-responsive Celiac Disease - More than 50% Continue to Ingest Gluten Unknowingly. 3. Jamma, Shailaja, MD, and Leffler, Daniel A, MD. “Nonresponsive Celiac Disease.” Real Life with Celiac Disease: AGA Press, 2010. 4. Medpage Today: ACG: More Celiac Disease May Be Refractory
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Celiac.com 08/20/2022 - It is not much of a reach to suspect additional food sensitivities in the context of celiac disease or gluten sensitivity. After all, celiac disease causes increased intestinal permeability(1) otherwise known as “leaky gut.” Since large, undigested gluten proteins can sometimes pass into the bloodstream, other food proteins are also likely to reach the circulation. The immune system reacts against such foreign proteins in an attempt to protect us. The presence of non-self proteins causes an immune system reaction just as if they were infectious microbes. And herein lies one answer to some, perhaps many, cases of incomplete recovery and refractory sprue. These conditions may sometimes be relatively easy to correct through the detection and avoidance of additional food sensitivities. Adult-diagnosed celiac patients have usually experienced many years of a leaky gut, with or without symptoms and ill health. Admittedly, these signs and symptoms can result from a variety of causes including nutritional deficiencies due to malabsorption, abnormal immune responses, damage to the protective mucosa of the intestinal wall resulting in a leaky gut, additional autoimmune conditions, and opportunistic infections. It sometimes seems that celiac disease just rolls out the red carpet for a host of additional ailments. Increased intestinal permeability, resulting in additional food allergies, is just one of the many contributors to this witches’ brew of additional ills that arise in untreated celiac disease and may continue despite careful avoidance of gluten. Considerable evidence has long pointed toward additional food allergies. Unfortunately, this information has largely been ignored. But recent developments in serological testing are now making it feasible, economical, and convenient, to identify and correct such food allergies. One article appeared almost thirty years ago in the peer reviewed literature reporting complete resolution of what was previously diagnosed as refractory sprue following removal of additional allergenic foods from the diet(2) . Another such publication documented the progress of one celiac patient who was thought to have refractory sprue. This individual recovered with the additional dietary exclusion of egg, chicken, and tuna(3) . This patient became very ill before the possibility of immune reactions to other dietary proteins was considered. More recent reports of the success of elemental diets in reversing refractory sprue further support this perspective(4) . Another group has indicated that 36% to 48% of celiac patients demonstrate antibody reactions to milk proteins(5) . Although there are some reports that the frequency of such sensitivities reduce with treatment time on a gluten-free diet(6,7), they also report a higher initial frequency of reactions to milk proteins. I have not heard of any new evidence suggesting that the injury to the intestinal mucosa caused by gluten can now be distinguished from similar injuries caused by milk protein allergies. Thus, any of a variety of food allergies might be contributing to such damage to the mucosa. The peer-reviewed reports cited above, along with the many posts to the Celiac Listserv indicating that additional food sensitivities are a factor in individual cases of celiac disease, suggest the need for vigilance among celiac patients, particularly those who are experiencing incomplete recovery on a strict gluten-free diet. Before leaping to the use of steroids, further antibody testing seems prudent. There are a number of commercial laboratories in the United States and at least one in the United Kingdom that offer IgG testing for delayed-type allergies to common foods. Although such tests are not perfect, they can provide valuable information for those who have not experienced a full recovery on a gluten-free diet, or some individuals who have been diagnosed with refractory sprue. The therapeutic use of systemic steroids can produce some very dangerous side effects. IgG blood testing and dietary exclusion of identified allergens, on the other hand, involves a simple, convenient test followed by the kind of dietary inconvenience that most of us are already well versed in. If possible, ELISA or similar testing ought to be done prior to beginning steroids, as such drugs may be unnecessary, or they may compromise the accuracy of the blood test. Sources: Pizzuti D, Bortolami M, Mazzon E, Buda A, Guariso G, D'Odorico A, Chiarelli S, D'Inca R, De Lazzari F, Martines D. Transcriptional downregulation of tight junction protein ZO-1 in active coeliac disease is reversed after a gluten-free diet. Dig Liver Dis. 2004 May;36(5):337-41. Baker AL, et al. Refractory sprue: recovery after removal of non-gluten dietary proteins. Ann Intern Med. 1978 Oct;89(4):505-8. Volta U, et al. Antibodies to dietary antigens in coeliac disease. Scand J Gastroenterol. 1986 Oct;21(8):935-40. Mandal A, Mayberry J. Elemental diet in the treatment of refractory coeliac disease. Eur J Gastroenterol Hepatol. 2001 Jan;13(1):79-80. Kemeny DM, Urbanek R, Amlot PL, Ciclitira PJ, Richards D, Lessof MH.Sub-class of IgG in allergic disease. I. IgG sub-class antibodies in immediate and non-immediate food allergy. Clin Allergy. 1986 Nov;16(6):571-81. Paranos S, et al. Lack of cross-reactivity between casein and gliadin in sera from coeliac disease patients. Int Arch Allergy Immunol. 1998 Oct;117(2):152-4. Scott H, et al. Immune response patterns in coeliac disease. Serum antibodies to dietary antigens measured by an enzyme linked immunosorbent assay (ELISA). Clin Exp Immunol. 1984 Jul;57(1):25-32.
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Celiac.com 12/28/2020 - With the outbreak of the SARS-CoV-2 virus, and cases of COVID-19 spreading worldwide, a number of people have been worried about celiac disease as a risk factor. A team of clinicians recently compiled a list of guidelines to help gastroenterologists and nutritionists in supporting their celiac disease patients during the COVID-19 outbreak. Among their insights, the team reminds readers that there is currently no data on the risk of COVID-19 and its outcomes in celiac disease. But, there is no evidence that celiac disease in itself represents a COVID-19 risk factor. Proven risk factors for COVID-19 remain old age, hypertension, diabetes, coronary artery disease, pulmonary disease, chronic kidney disease, and high body mass index. Depending on local recourses, the team encourages clinicians managing celiac patients during Covid-19 to initiate a rapid online service to address the patients’ doubts about a gluten-free diet, along with the use of POC tests for urinary gluten peptides and serological antibodies. One potential impact of COVID-19 restrictions can be reduced access to gluten-free food, which celiacs require as treatment. The paper provides helpful advice on this, and numerous other topics, including: What about hyposplenism? The team advise doctors to reassure patients that functional hyposplenism does not pose any greater risk for Covid-19. Refractory celiac disease Patients with refractory celiac disease and/or taking immunosoppressive/chemotherapic agents could face a higher risk for COVID-19, and so they should be vigilant about social distancing and shielding. Telecon clinics Telemedicine and gastroenterological/nutritional video-consulting is very helpful to patients with celiac disease. Dietary advice including Mediterranean and gluten-free dietary regimens The paper offers helpful tips on improving patient diet, especially by following a gluten-free Mediterranean diet, and consuming more antioxidant micronutrients. Read the full recommendations in BMC Gastroenterology volume 20, Article number: 387 (2020) The clinicians team included Luca Elli, Donatella Barisani, Valentina Vaira, Maria Teresa Bardella, Matilde Topa, Maurizio Vecchi, Luisa Doneda, Alice Scricciolo, Vincenza Lombardo & Leda Roncoroni. They are variously affiliated with the Center for Prevention and Diagnosis of Celiac Disease, Gastroenterology and Endoscopy Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy; the Department of Pathophisiology and Transplantation, University of Milano, Milan, Italy; the School of Medicine and Surgery, University of Milano-Bicocca, Monza, Italy; the Department of Pathophisiology and Transplantation, University of Milano, Milan, Italy; the Division of Pathology, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy; the Center for Prevention and Diagnosis of Celiac Disease, Gastroenterology and Endoscopy Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Via F. Sforza 35, 20122, Milan, Italy; and the Department of Biomedical, Surgical and Dental Sciences, University of Milan, Milan, Italy.
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Celiac.com 04/22/2020 - Collagenous sprue, aka enteritis, is a rare mucosal small intestinal disorder marked by a clear histopathological lesion containing collagen. People who suffer from collagenous sprue frequently experience severe diarrhea, progressive nutrient malabsorption, protein depletion and weight loss. Closely linked to celiac disease, collagenous sprue is notoriously difficult to treat, and medical literature records only a few claims of successful therapy. Clinical studies suggest myriad causes for collagenous sprue, with prognoses often depending on the cause. Recently, collagenous sprue has been found in numerous diverse settings, including as a paraneoplastic feature of early malignancies, and as a result of the toxic medications including non-steroidal anti-inflammatory drugs (NSAIDs) and the angiotensin II receptor antagonist, olmesartan. In a paper published in the International Journal of Celiac Disease, Hugh James Freeman, of the Department of Medicine (Gastroenterology), University of British Columbia, Vancouver, BC, Canada, writes about the return to normal of such clinical and pathological changes, and notes that knowing the medication history of such patients is crucial to treating patients with sprue-like intestinal disease. Knowing the patient medication history can provide a crucial roadmap for treating such patients. Read more in the International Journal of Celiac Disease. 2019, 7(1), 13-15. DOI: 10.12691/ijcd-7-1-2
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Celiac.com 06/14/2018 - Refractory celiac disease type II (RCDII) is a rare complication of celiac disease that has high death rates. To diagnose RCDII, doctors identify a clonal population of phenotypically aberrant intraepithelial lymphocytes (IELs). However, researchers really don’t have much data regarding the frequency and significance of clonal T cell receptor (TCR) gene rearrangements (TCR-GRs) in small bowel (SB) biopsies of patients without RCDII. Such data could provide useful comparison information for patients with RCDII, among other things. To that end, a research team recently set out to try to get some information about the frequency and importance of clonal T cell receptor (TCR) gene rearrangements (TCR-GRs) in small bowel (SB) biopsies of patients without RCDII. The research team included Shafinaz Hussein, Tatyana Gindin, Stephen M Lagana, Carolina Arguelles-Grande, Suneeta Krishnareddy, Bachir Alobeid, Suzanne K Lewis, Mahesh M Mansukhani, Peter H R Green, and Govind Bhagat. They are variously affiliated with the Department of Pathology and Cell Biology, and the Department of Medicine at the Celiac Disease Center, New York Presbyterian Hospital/Columbia University Medical Center, New York, USA. Their team analyzed results of TCR-GR analyses performed on SB biopsies at our institution over a 3-year period, which were obtained from eight active celiac disease, 172 celiac disease on gluten-free diet, 33 RCDI, and three RCDII patients and 14 patients without celiac disease. Clonal TCR-GRs are not infrequent in cases lacking features of RCDII, while PCPs are frequent in all disease phases. TCR-GR results should be assessed in conjunction with immunophenotypic, histological and clinical findings for appropriate diagnosis and classification of RCD. The team divided the TCR-GR patterns into clonal, polyclonal and prominent clonal peaks (PCPs), and correlated these patterns with clinical and pathological features. In all, they detected clonal TCR-GR products in biopsies from 67% of patients with RCDII, 17% of patients with RCDI and 6% of patients with gluten-free diet. They found PCPs in all disease phases, but saw no significant difference in the TCR-GR patterns between the non-RCDII disease categories (p=0.39). They also noted a higher frequency of surface CD3(−) IELs in cases with clonal TCR-GR, but the PCP pattern showed no associations with any clinical or pathological feature. Repeat biopsy showed that the clonal or PCP pattern persisted for up to 2 years with no evidence of RCDII. The study indicates that better understanding of clonal T cell receptor gene rearrangements may help researchers improve refractory celiac diagnosis. Source: Journal of Clinical Pathologyhttp://dx.doi.org/10.1136/jclinpath-2018-205023
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