Immunoproliferative small intestinal disease or celiac disease.
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Adult↗
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Jejunal bacterial flora in chronic small bowel disease. I. Celiac disease. II. Regional enteritis.
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Bacteroides↗
Prevalence of celiac disease among relatives of sib pairs with celiac disease in U.S. families.
OBJECTIVE: Celiac disease is a familial malabsorptive disorder with an estimated prevalence in first-degree relatives of 10-12%. The prevalence for first-degree and more distant relatives has not been determined in families where there are two affected first-degree relatives. The aim of our investigation was to estimate the prevalence and relative risk for celiac disease in relatives of two siblings diagnosed with celiac disease. METHODS: We ascertained sib pairs with celiac disease, and then identified all living first-degree relatives and available second-degree relatives to minimize ascertainment bias. We measured IgA endomysial antibodies, a highly specific and sensitive assay for celiac disease, in all subjects without a confirmed biopsy diagnosis. For those individuals with positive serologic tests, IgA tissue transglutaminase antibody tests and human leukocyte antigen DQA1 and DQB1 genotyping were performed for additional confirmation. Individuals with positive biopsy and/or serology were considered affected. We calculated the relative risk of being affected with celiac disease using the lambda(R) statistic. RESULTS: The prevalence of celiac disease in relatives of affected sib pairs was as follows: 21.3% (13/61) of siblings (lambda(S) = 53); 14.7% (10/68) of offspring (lambda(O) = 37); 17.2% (28/163) of first-degree relatives; 19.5% (16/82) of second-degree relatives; and 17.8% (52/292) of all relatives (lambda(R) = 44.5). CONCLUSIONS: In these families, we identified a sibling risk approximately double that found in previous reports, as well as significant risk for more distant relatives, probably because of sharing of a common gene. In families where at least two siblings have been diagnosed with celiac disease, relatives are at high risk for celiac disease. Screening should be considered for all family members.
Autoantibodies↗
The prevalence of celiac disease among family members of celiac disease patients.
INTRODUCTION: Celiac disease (CD) is more common in certain risk groups. Family members of known celiac patients represent the most important group. Serological screening enables us to detect patients before they develop serious complications. HLA typing has also proven to be a valuable diagnostic tool, especially in excluding the disease. METHODS: To assess the prevalence of CD among family members, we screened 106 first-degree relatives (73 parents, 33 siblings; mean age 27.9 years) of 45 celiac patients in NE Slovenia. We analysed antigliadin (AGA) and antiendomysium (EMA) antibodies. Levels of IgG and IgAAGA were determined using the ELISA method, and EMA using indirect immunofluorescence. Serologically positive patients were recalled for intestinal biopsy and were HLA typed. Intestinal biopsy was performed by peroral aspiration capsule or during upper GI endoscopy. Biopsy specimens were examined histologically. RESULTS: Six family members (5.67%) were both AGA IgG and EMA positive, and one (0.94%) was only EMA positive. All were either HLA DQ2 or DQ8 positive. Nine family members (8.49%) were only AGA IgG positive, two of them lacked the HLA DQ susceptibility alleles. Intestinal biopsy was performed in six family members, and the diagnosis of CD confirmed in five. All were both AGA IgG and EMA positive. They were either symptom-free or had only mild gastrointestinal symptoms, and carried the known HLA DQ risk alleles. The minimum prevalence of CD among family members in NE Slovenia can therefore be estimated at 4.72%. DISCUSSION: The prevalence of CD among first-degree relatives is much higher than the prevalence of the disease in the general population. Most of these patients have an atypical form of the disease and would therefore be overlooked without an active search. Serological testing is recommended for all first-degree relatives of CD patients; they should also undergo HLA typing to detect those whose HLA phenotype is consistent with CD. This approach can also help in excluding individuals who do not need further diagnostic procedures for CD.
Adolescent↗
Association of Crohn's disease and latent celiac disease: a case report and review of the literature.
BACKGROUND: Crohn's disease is a chronic inflammatory disease of the intestine potentially affecting all parts of the intestine with predilection sites in the terminal ileum and proximal colon. Its prevalence in Western Europe is 20-40/100,000 with equal affection of both sexes and familiar accumulation. Histopathologically, it is characterized by a discontinuous, segmental manifestation and implication of all intestinal layers. Celiac disease, on the other hand, is defined by histologically proven villous atrophy associated with hyperplasia of crypts, lymphocytic infiltration and clinical improvement after a gluten-free diet. CASE REPORT: We report the case of a 52-year-old man presenting with long-term diarrhea and loss of weight associated with Crohn's disease. After interventional therapy for an unstable coronary artery syndrome and medical therapy for hyperthyroidism, the diarrhea stopped only after maintaining a gluten-free diet. A latent form of celiac disease (clinical symptoms, improvement after gluten-free diet, detection of anti-gliadin IgA antibodies, negative histology) was diagnosed. CONCLUSION: To our knowledge, this is the first report on the association of Crohn's disease and the latent form of celiac disease in the same patient. Whereas in most cases, Crohn's disease develops secondary to a pre-existing celiac disease, in our patient, latent celiac disease was diagnosed years after the onset of and therapy for Crohn's disease.
Celiac Disease↗
[Gluten-free diet in therapy of celiac disease; two cases of celiac disease treated with gluten-free diet].
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Celiac Disease↗
[The role of connective tissue in the morphology and function of intestinal mucosa. Its participation in the pathogenesis of celiac diseases].
The differentiation, architecture and function of the mucosa of the digestive tract is the result of close interactions between the epithelial cells and their underlying connective tissue. This interaction not only plays a role in the normal morphological organization of the intestinal mucosa but also in the organization and maturation of the enterocytes, the development of some of their enzymatic activities, the transport of nutrients and the restitution of the epithelium following denudation of the surface of villi due to loss of enterocytes. It is thought that disturbances of the cells of the connective tissue, especially of the myofibroblasts as well as of some of the above mentioned mechanisms, participate in the pathogenesis of some gastrointestinal diseases, celiac disease among them. This review summarizes some of the knowledge in this area.
Basement Membrane↗
Transglutaminase-catalyzed reactions responsible for the pathogenesis of celiac disease and neurodegenerative diseases: from basic biochemistry to clinic.
Transglutaminases (TGases) are enzymes which catalyze the cross linking of a glutaminyl residue of a protein/peptide substrate to a lysyl residue of a protein/peptide co-substrate with the formation of an N-gamma-(epsilon-L-glutamyl)-L-lysine [GGEL] cross link (isopeptidic bond) and the concomitant release of ammonia. Such cross-linked proteins are often highly insoluble. The TGases are closely related enzymes and can also catalyze other important reactions for cell life. Recently, several findings concerning the relationships between the biochemical activities of the TGases and the basic molecular mechanisms responsible for some human diseases, have been reported. For example, some neurodegenerative diseases, such as Alzheimer's disease (AD), Huntington's disease (HD), Parkinson's disease (PD), supranuclear palsy, etc., are characterized in part by aberrant cerebral TGase activity and by increased cross-linked proteins in affected brains. Our article describes the biochemistry and the physio-pathological roles of the TGase enzymes, with particular reference to human pathologies in which the molecular mechanism of disease can be due to biochemical activities of the tissue TGase enzyme (tTGase, type 2), such as in a very common human disease, Celiac Disease (CD), and also in certain neuropsychiatric disorders.
Brain↗
Pregnancy outcome of patients with known celiac disease.
OBJECTIVE: Celiac disease is a permanent intolerance to gluten, probably induced by an autoimmune mechanism. Controversy exists regarding the association between celiac disease and infertility, abortions, intra-uterine growth restriction (IUGR) and stillbirths. The present study was designed to investigate pregnancy outcome of patients with celiac disease. METHODS: A retrospective comparison between all pregnancies of women with and without known celiac disease, delivered during the years 1988-2002, was conducted. RESULTS: During the study period there were 48 deliveries of patients with celiac disease and 143,663 pregnancies of patients without known celiac disease. No statistically significant differences were noted between the groups regarding maternal or perinatal outcomes, including fertility treatments (0% among patients with known celiac versus 2.5% among patients without known celiac sprue; p=0.267), recurrent abortions (0 versus 5.2%; p=0.103), perinatal mortality (2.1 versus 1.4%; p=0.668). However, higher rates of labor induction (29.2 versus 11.9%; p<0.001) and IUGR (6.3 versus 2.1%; p=0.042) were found among patients with celiac disease as compared to patients without known celiac disease. CONCLUSION: The course of pregnancy of patients with celiac disease including perinatal outcomes is favorable. Since we found higher rates of IUGR, careful surveillance should be performed for early detection of IUGR. Further, prospective studies should focus on screening for celiac disease among patients presenting with IUGR of an unknown etiology.
Adult↗
The molecular basis for oat intolerance in patients with celiac disease.
BACKGROUND: Celiac disease is a small intestinal inflammatory disorder characterized by malabsorption, nutrient deficiency, and a range of clinical manifestations. It is caused by an inappropriate immune response to dietary gluten and is treated with a gluten-free diet. Recent feeding studies have indicated oats to be safe for celiac disease patients, and oats are now often included in the celiac disease diet. This study aimed to investigate whether oat intolerance exists in celiac disease and to characterize the cells and processes underlying this intolerance. METHODS AND FINDINGS: We selected for study nine adults with celiac disease who had a history of oats exposure. Four of the patients had clinical symptoms on an oats-containing diet, and three of these four patients had intestinal inflammation typical of celiac disease at the time of oats exposure. We established oats-avenin-specific and -reactive intestinal T-cell lines from these three patients, as well as from two other patients who appeared to tolerate oats. The avenin-reactive T-cell lines recognized avenin peptides in the context of HLA-DQ2. These peptides have sequences rich in proline and glutamine residues closely resembling wheat gluten epitopes. Deamidation (glutamine-->glutamic acid conversion) by tissue transglutaminase was involved in the avenin epitope formation. CONCLUSIONS: We conclude that some celiac disease patients have avenin-reactive mucosal T-cells that can cause mucosal inflammation. Oat intolerance may be a reason for villous atrophy and inflammation in patients with celiac disease who are eating oats but otherwise are adhering to a strict gluten-free diet. Clinical follow-up of celiac disease patients eating oats is advisable.
Atrophy↗
[Case report: celiac disease].
INTRODUCTION: Celiac disease (nontropical sprue, gluten-sensitive enteropathy, chronic intestinal malabsorption disorder) is caused by gluten intolerance. This hereditary disorder is caused by sensitivity to gliadin. Because the body's own immune system causes the damage, celiac disease is considered to be an autoimmune disorder. However, it is also classified as a disease of malabsorption because nutrients are not absorbed. When people with celiac disease eat foods containing gluten, their immune system responds by damaging the small intestine. Specifically, tiny finger-like protrusions, called villi, on the lining of the small intestine are lost. The diagnosis is suspected on the basis of symptoms and signs, enhanced by laboratory and x-ray studies, and confirmed by biopsy revealing flat mucosa and subsequent clinical and histologic improvement on a gluten-free diet. Gluten must be excluded from diet. Supplementary vitamins, minerals and hematinics may be given depending on deficiency. CASE REPORT: This is a case report of a 23-year old female patient with a mineralization defect (osteomalacia) and secondary osteoporosis caused by long-time unrecognized celiac disease. The patient had many symptoms: short stature, steatorrhea, anemia, weight loss and chronic bone pain. Laboratory and x-ray studies and jejunal biopsy revealed a chronic intestinal malabsorption disorder caused by gluten intolerance. Gluten-free diet and supplementary vitamins, minerals and hematinics were included with apparent clinical remission. DISCUSSION AND CONCLUSION: Some people with celiac disease may not have symptoms. The undamaged part of their small intestine is able to absorb enough nutrients to prevent symptoms. However, people without symptoms are still at risk for complications of celiac disease. Biopsy of the small intestine is the best way to diagnose celiac disease. Decreased bone density (osteoporosis and osteomalacia) is a serious problem for celiacs. If calcium is not absorbed, due to small intestinal damage caused by untreated celiac disease, bones are not as dense. The only treatment for celiac disease is gluten-free diet, that is, avoiding all foods that contain gluten. For most people, this diet stops symptoms, heals the existing intestinal damage, and prevents further damage.
Adult↗
Allergy prevalence in adult celiac disease.
BACKGROUND: Celiac disease is considered to arise from an inappropriate T-cell-mediated immune response against ingested gluten in genetically predisposed people, whereas the T(H)2-type lymphocytes are mostly involved in IgE-mediated reactions. The matter of possible coexistence of T(H)1- and T(H)2-type diseases is still debated. OBJECTIVE: This study was aimed to evaluate the allergy prevalence in a large series of adults with untreated celiac disease and their families at the moment of diagnosis. We also evaluated whether 1 year of gluten-free diet had any effect on allergy prevalence in our cohort. METHODS: At the moment of celiac disease diagnosis a standardized questionnaire was administered for detailed information on presence and type of any allergy symptoms in 1044 adult patients with celiac disease, 2752 relatives, and 318 spouses. Those reporting any allergy underwent tests with dosage of serum levels of total IgE and search for serum specific IgE with a standard makeup of 20 antigens and PRICK tests in selected individuals. At follow-up visit patients with celiac disease were administered the same allergy questionnaire. RESULTS: One hundred seventy-three patients with celiac disease (16.6%), 523 relative (19%), and 43 spouses (13.5%) had at least 1 allergy (P=not significant). Atopic dermatitis was more frequent in patients with celiac disease (3.8%) and their relatives (2.3%) than in spouses (1.3%). The presence of allergy in general and atopic dermatitis was not affected by presence of overt malabsorption or duration of undiagnosed disease. Follow-up data showed no change in allergy prevalence in the cohort examined. CONCLUSION: Allergy prevalence in a large series of patients with celiac disease is not different from that of their relatives and spouses. However, atopic dermatitis was about 3 times more frequent in patients with celiac disease and 2 times more frequent in their relatives than in spouses. One year of gluten-free diet did not change allergy prevalence in the celiac group under investigation.
Adult↗
Adult celiac disease presented with celiac crisis: severe diarrhea, hypokalemia, and acidosis.
An acute severe onset of celiac disease is very uncommon in adults. We describe a patient with adult celiac disease who presented with acute diarrhea that lead rapidly to a life threatening hypokalemia and acidosis, the so-called celiac crisis. Celiac crisis, described mainly in children younger than two years of age, has become very rare due to earlier diagnosis and effective therapy of the disease. The case described is an example of the heterogeneous clinical course of celiac disease and emphasizes the need to consider it in the differential diagnosis, even in adults suffering from acute diarrhea and acidosis.
Acidosis↗
Risk of non-Hodgkin lymphoma in celiac disease.
CONTEXT: Celiac disease is one of the most common lifelong disorders. Non-Hodgkin lymphoma is a possible complication of celiac disease and may lead to a large portion of lymphoma cases. OBJECTIVE: To quantify the risk for developing non-Hodgkin lymphoma of any primary site associated with celiac disease. DESIGN AND SETTING: Multicenter, case-control study conducted between January 1996 and December 1999 throughout Italy. PATIENTS: Cases were older than 20 years (median, 57; range, 20-92 years) with non-Hodgkin lymphoma of any primary site and histological type and were recruited at the time of the diagnosis. Controls were healthy adults (2739 men and 2981 women) from the general population. MAIN OUTCOME MEASURE: Positive test result for class A serum antiendomysial antibody. RESULTS: Celiac disease was diagnosed in 6 (0.92%) of 653 patients with lymphoma. Of the 6 cases, 3 were of B-cell and 3 were of T-cell origin. Four of 6 cases had lymphoma primarily located in the gut. In the control group, 24 (0.42%) had celiac disease. The odds ratio (adjusted for age and sex) for non-Hodgkin lymphoma of any primary site associated with celiac disease was 3.1 (95% confidence interval [CI], 1.3-7.6), 16.9 (95% CI, 7.4-38.7) for gut lymphoma, and 19.2 (95% CI, 7.9-46.6) for T-cell lymphoma, respectively. The risk for non-Hodgkin lymphoma for the overall population, which was adjusted for age and sex, was 0.63% (95% CI, - 0.12% to 1.37%). CONCLUSION: Celiac disease is associated with an increased risk for non-Hodgkin lymphoma, especially of T-cell type and primarily localized in the gut. However, the association does not represent a great enough risk to justify early mass screening for celiac disease.
Adult↗
Gender and clinical presentation in adult celiac disease.
BACKGROUND: Celiac disease may present in various forms. This study aimed to investigate whether gender affects the clinical presentation of the disease in adult celiac patients from the Mediterranean area. METHODS: This study retrospectively analyzes data collected in all adult patients with celiac disease (n = 195) seen during the past 13 years at the Gastrointestinal Unit of the Federico II University of Naples, Italy. RESULTS: In these series of patients the ratio of women to men was 3.33. Age at diagnosis was lower in women that in men (p < 0.05). Except for asthenia, all signs and symptoms were more frequent in women than in men. Hypochromic anemia was the most commonest finding in women and was 40% more frequent in women than in men (p < 0.001). Dyspepsia was twice as frequent in women as in men (p < 0.05); genital disorders were reported by 44% of women and by no men. Recent weight loss or low body mass index was the commonest finding in men. About 60% of men and women reported diarrhea; among patients without diarrhea, the prevalence of hypochromic anemia differed between sexes (p < 0.05), occurring in about 80% of women. CONCLUSION: This study shows that the clinical presentation of celiac disease is not the same in men and women. The disease is not only more frequent in women than in men but is also more severe and more rapid. The data also suggest the need to look for celiac disease in patients with unexplained hypochromic anemia.
Adult↗
Molecular mechanisms of the adaptive, innate and regulatory immune responses in the intestinal mucosa of celiac disease patients.
Celiac disease is a complex genetic disorder that affects the small intestine of genetically predisposed individuals when they ingest gluten, a dietary protein. Although several genome screens have been successful in identifying susceptibility loci in celiac disease, the only genetic contributors identified so far are the human leukocyte antigen (HLA)-DQ2/DQ8 molecules. One of the most important aspects in the pathogenesis of celiac disease is the activation of a T-helper 1 immune response, when the antigen-presenting cells that express HLA-DQ2/DQ8 molecules present the toxic gluten peptides to reactive CD4(+) T-cells. Recently, new insights into the activation of an innate immune response have also been described. It is generally accepted that the immune response triggers destruction of the mucosa in the small intestine of celiac disease patients. Hence, the activation of a detrimental immune response in the intestine of celiac disease patients appears to be key in the initiation and progression of the disease. This review summarizes the immunologic pathways that have been studied in celiac disease thus far, and will point to new potential candidate genes and pathways involved in the etiopathogenesis of celiac disease, which should lead to novel alternatives for diagnosis and treatment.
Adaptation, Physiological↗
Duration of exposure to gluten and risk for autoimmune disorders in patients with celiac disease. SIGEP Study Group for Autoimmune Disorders in Celiac Disease.
BACKGROUND & AIMS: The relationship between celiac disease and many autoimmune disorders has been explained by the sharing of a common genetic factor. In a multicenter national study, we examined the relationship between the prevalence of autoimmune disorders in celiac disease and the duration of exposure to gluten. METHODS: Over a 6-month period, 909 patients with celiac disease (group A; mean age, 16.1 +/- 3.8 years; grouped according to age at diagnosis into three subgroups [group A1, <2 years; group A2, 2-10 years; and group A3, >10 years]), 1268 healthy controls (group B; mean age, 20.8 +/- 4.5 years), and 163 patients with Crohn's disease (group C; mean age, 28.8 +/- 10 years) were evaluated for the presence of autoimmune disorders. RESULTS: Prevalence of autoimmune disorders in group A was significantly higher than in group B (14% vs. 2.8%; P < 0.000001) but not higher than in group C (12.9%). Prevalence of autoimmune disorders in celiac disease increased with increasing age at diagnosis: 5.1% in group A1, 17% in group A2, and 23.6% in group A3 (P = 0.000001). In group A3, the prevalence of autoimmune disorders was significantly higher than in group C. In a logistic regression model, age at diagnosis was the only significant predictor variable of the odds of developing an autoimmune disorder (r = 0.3; P < 0.000001). CONCLUSIONS: Our data show for the first time that the prevalence of autoimmune disorders in celiac disease is related to the duration of exposure to gluten.
Adolescent↗
Urinary NOx:creatinine ratios during gluten challenge in children with celiac disease.
OBJECTIVES: Celiac disease is a gluten-induced small bowel enteropathy. Inflammation is known to be associated with enhanced nitric oxide (NO) production. An increase in urinary nitrate and nitrite (NOx) reflects increased NO production. The urinary NOx:creatinine ratio can be used as an indicator of the endogenous NO production. The aim of the study was to determine whether the urinary NOx:creatinine ratio of celiac disease patients increases during gluten challenge. METHODS: The authors studied 20 patients with unconfirmed celiac disease who had been following a gluten-free diet for at least 1 year. These patients underwent an 80-day gluten challenge. Urinary samples were obtained before and 10, 20, 40, and 80 days after starting the gluten challenge. The Griess reagent method was used for measuring urinary NOx. RESULTS: Gluten challenge confirmed the diagnosis of celiac disease in 15 of 20 patients. The NOx:creatinine ratios (mmol:mmol) of the biopsy-confirmed celiac disease patients were significantly higher than those of the unconfirmed celiac disease patients (0.67 vs. 0.17 on day 10; 0.78 vs. 0.15 on day 20; 0.85 vs. 0.25 on day 40; and 0.85 vs. 0.17 on day 80). CONCLUSIONS: Gluten challenge resulted in an increased urinary NOx:creatinine ratio in patients with biopsy-confirmed celiac disease. The NOx:creatinine ratio could be useful for the serial evaluation of disease activity.
Celiac Disease↗