[Etiology and pathogenesis of celiac disease. I. Behavior of gamma-glutamyl transpeptidase at different stages of gluten-dependent celiac disease].
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Celiac Disease↗
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Positive tissue transglutaminase antibodies with negative endomysial antibodies: low rate of celiac disease.
BACKGROUND: Screening for celiac disease is based on the sequential evaluation of serologic tests and intestinal biopsy; an optimal screening protocol is still under investigation. The screening policy of one of the main healthcare providers in Israel (Maccabi) consists of measuring total immunoglobulin A and tissue transglutaminase IgA antibodies and confirming positive results by endomysial antibodies. For IgA-deficient patients antigliadin IgG is measured. OBJECTIVES: To evaluate the use of tTGA as a first-level screening test in patients suspected of having celiac disease METHODS: The results of tTGA and EMA tests over a 3 month period were obtained from the laboratory computer. Letters were sent to the referring physicians of patients with positive tests, requesting clinical information and small intestinal biopsy results. tTGA was performed using an anti-guinea pig tTG-IgA enzyme-linked immunosorbent assay kit. RESULTS: Overall, 2,505 tTGA tests were performed: 216 (8.6%) were tTGA-positive of which 162 (75%) were EMA-negative (group 1) and 54 (25%) EMA-positive (group 2.) Clinical information was obtained for 91 patients in group 1 and 32 in group 2. Small intestinal biopsy was performed in 33 (36%) and 27 patients (84%) in groups 1 and 2, respectively. Celiac disease was diagnosed in 4 biopsies (12%) in group 1 and 23 (85%) in group 2 (P < 0.0001). The positive predictive value was 45% for tTGA and 85% for EMA. CONCLUSIONS: Symptomatic patients with positive tTGA and negative EMA have a low rate of celiac disease compared to those who are tTGA-positive and EMA-positive. Confirmation with EMA is advised when tTGA is performed as a first-level screening for suspected celiac disease.
Adolescent↗
Enhanced peptide-binding capacities of small intestinal brush border membranes in celiac disease.
In pathogenesis of celiac disease, the significance of prolamin peptide interactions with enterocytes is controversial. Changes in cellular metabolism induced by gliadin peptides, as well as uptake and presentation by enterocytes, are discussed. We analyzed peptide binding to enterocytic membranes as a potential key event. Binding capacities of brush border membranes isolated from small intestinal biopsies of untreated (n = 49) and treated celiac patients on a gluten-free diet (n = 30), as well as control subjects (n = 43), were measured with a dot blot chemiluminescence assay. Synthetic gliadin peptides comprising amino acid position 8-19 (G XIV) and 30-41 (G XI) of alpha-gliadins, a peptic-tryptic digest of gliadin (PT-GLI), and a synthetic zein peptide were used. Comparing treated celiac patients with controls, we observed significantly enhanced membrane-binding of PT-GLI [mean 122.4 densitometric units/microg (95% confidence interval 116.0-128.9) vs 108.9 (102.1-115.7)] and of zein peptide [50.2 (38.4-61.9) vs 28.8 (13.4-44.2)], but only slightly increased binding of the synthetic gliadin peptides G XIV [65.5 (60.6-70.5) vs 62.4 (56.3-68.5) and G XI [75.2 (69.8-80.6) vs 65.9 (55.2-76.5)]. Independent of patient group, membrane-binding capacities for celiac-active gliadin peptides exceeded those of the zein peptide. Thus, interaction of gliadin peptides with the apical enterocytic membrane was not found exclusively in celiac disease. Furthermore, increased binding capacities in treated celiac disease were not confined to celiac-active peptides. Quantitative differences in gliadin peptide binding as a primary characteristic in celiac disease might contribute to pathogenetic effects exerted on small intestinal epithelial cells.
Adolescent↗
Prevalence of celiac disease in dyspeptic patients.
BACKGROUND: Celiac disease is one of the most common dietary-mediated inflammatory enteropathies that occur in genetically predisposed individuals in response to gluten intolerance. This disorder has become more common than in the past, even if it frequently remains undetected for long periods of time. The screening of patients with dyspepsia, a symptom that can be a manifestation of celiac disease, may allow an early identification of affected individuals. Endoscopy and serological tests may have an important role in the management of these patients. AIMS: Determining the prevalence of celiac disease in dyspeptic patients submitted to routine diagnostic upper gastrointestinal endoscopy. PATIENTS/METHODS: Endoscopic findings, duodenal biopsy histological specimens and serological test results were assessed and compared in 142 patients consecutively admitted with dyspeptic symptoms between October 2001 and October 2003. RESULTS: An endoscopic pattern suggestive of celiac disease was observed in four patients. The IgG-AGA assay was positive in 24 patients. Two of the IgG-AGA positive patients also yielded positive results on the IgA-EMA test and concomitantly disclosed endoscopic pattern and histological features in duodenal biopsy compatible with celiac disease. Abnormal endoscopic findings were notably marked in biopsy proven celiac patients. Therefore, a 1.4% prevalence of celiac disease was observed in this study group. CONCLUSIONS: The high prevalence of celiac among dyspeptic symptomatic individuals indicates that they are a higher risk group for developing celiac disease. Undiagnosed celiac disease may be inferred by endoscopic markers of duodenal villous atrophy. Endoscopic findings, however, may be inadequate to suitably diagnose this disease and consequently the incorporation of diagnostic serologic assays of celiac disease in routine testing for dyspepsia is strongly recommended.
Adolescent↗
The prevalance of celiac disease in West Virginia.
Celiac disease (CD) has been long considered as a "European disease" that is rarely seen in the North America. Recent data has refuted this notion and suggested that celiac disease in the United States is as common as in Europe. The atypical clinical presentation of celiac disease was one of the major reasons implicated for the under-diagnosis of this disease in American children. In this report, we describe several case presentations of children with celiac disease in order to update primary care physicians on the pathophysiology, diagnosis, treatment, and prognosis of this increasingly prevalent disease.
Celiac Disease↗
HLA-DQA1 and HLA-DQB1 genetic markers and clinical presentation in celiac disease.
BACKGROUND: Patients with celiac disease are diagnosed at any age and can exhibit a wide range of clinical manifestations. The reasons for this are unclear. The aim of this study was to investigate a possible correlation between the HLA-DQA1 and HLA-DQB1 genetic markers and clinical features of celiac disease. METHODS: A total of 133 patients with celiac disease were tested for the HLA-DQA1 and HLA-DQB1 genes. Their corresponding allele and haplotype frequency distributions were estimated from the phenotypes found. The results were correlated with data from the clinical records. RESULTS: The DQ2 molecule was found in 93% of the patients, and DQ2 or DQ8 was found in 98%. The DQA1*0201-DQB1*0202 haplotype showed strong linkage disequilibrium. DQ2 homozygosis was significantly associated with female sex, earlier age at diagnosis, and shorter delay between onset of symptoms and diagnosis. Double-dose DQB1*02 (01-02) allele was more frequent in patients with the classic presentation of the disease. CONCLUSIONS: The genetic markers investigated may prove useful for diagnosing and managing celiac disease. With some clinical variables, correlations not previously described were found. These correlations have a moderate strength and, therefore, must be confirmed by other studies.
Adolescent↗
Capsule endoscopy in celiac disease: diagnosis and management.
Celiac disease occurs in about 1% of the population. Although diarrhea is the classical presentation, the diagnosis of celiac disease is frequently not considered as part of the differential diagnosis of a variety of different symptoms. It is,therefore, imperative that physicians who perform capsule endoscopy, and those who review the images, are aware of the variety of mucosa appearances inpatients who have celiac disease. In addition, studies are needed to determine the role of capsule endoscopy in the diagnosis and management of celiac disease.
Celiac Disease↗
Free perforation due to intestinal lymphoma in biopsy-defined or suspected celiac disease.
Malignant complications occur in celiac disease and dermatitis herpetiformis, particularly small bowel lymphoma. Although intestinal ulceration, especially in jejunum or ileum is rare, spontaneous free perforation of the intestine developed in 6 patients with established or suspected celiac disease and dermatitis herpetiformis due to a malignant lymphoma. In 3 cases, perforation developed after celiac disease was diagnosed and compliance with a gluten-free diet was strictly maintained and repeatedly evaluated. In 1 of these cases, refractory celiac disease was defined. In another, free perforation was the presenting manifestation of the lymphoma and this led to a later diagnosis of subsequent biopsy-defined latent celiac disease. In 2 others, celiac disease was suspected but the rapidly deteriorating clinical course prohibited institution of a gluten-free diet. In all patients, the lymphoma involved the jejunum, but in 1 of these, perforation was defined in the colon indicating that any intestinal site may suffer free perforation from lymphoma in celiac disease. This report documents a high mortality for this serious complication in celiac disease, its lack of predictability and the potential for development even with strict compliance to a gluten-free diet.
Adult↗
Recognition and management of the cutaneous manifestations of celiac disease: a guide for dermatologists.
In celiac disease, the ingestion of gluten-containing cereals, such as wheat, rye, and barley, results in small-bowel mucosal inflammation and villous atrophy with crypt hyperplasia. The prevalence of the condition may be as high as 1% in the adult population. The disease can also embrace various extraintestinal manifestations, of which dermatitis herpetiformis is the best known. Earlier, dermatitis herpetiformis was considered a skin disease occurring often concomitantly with celiac disease. At present, a body of evidence shows that dermatitis herpetiformis is a cutaneous manifestation of celiac disease, and affects approximately 25% of patients with celiac disease. Both conditions can appear in the same family and are closely linked to HLA class II locus in chromosome 6; 90% of patients have HLA DQ2 and, almost all the remainder, HLA DQ8. All patients with dermatitis herpetiformis have at least some-degree of mucosal inflammation or lesion consistent with celiac disease. The etiology of celiac disease in not fully understood, but tissue transglutaminase seems to be the predominant autoantigen both in the intestine and the skin. Serum antibodies against tissue transglutaminase can be used in the serologic screening and follow-up of dietary compliance of patients with celiac disease. Gluten-free diet is essential in the treatment of both conditions, and oral dapsone is usually needed in newly detected dermatitis herpetiformis in order to alleviate symptoms. Oral mucosal lesions, alopecia areata, and vitiligo probably occur more frequently in patients with dermatitis herpetiformis than in the general population. By contrast, the reported association of celiac disease with psoriasis seems to be coincidental.
Anti-Inflammatory Agents, Non-Steroidal↗
Refining the rules of gliadin T cell epitope binding to the disease-associated DQ2 molecule in celiac disease: importance of proline spacing and glutamine deamidation.
Celiac disease is driven by intestinal T cells responsive to proline-rich gluten peptides that often harbor glutamate residues formed by tissue transglutaminase-mediated glutamine conversion. The disease is strongly associated with the HLA variant DQ2.5 (DQA1*05, DQB1*02), and intestinal gluten-reactive T cells from DQ2.5-positive patients are uniquely restricted by this HLA molecule. In this study, we describe the mapping of two novel T cell epitopes of gamma-gliadin and the experimental identification of the DQ2.5 binding register of these and three other gamma-gliadin epitopes. The new data extend the knowledge base for understanding the binding of gluten peptides to DQ2.5. The alignment of the experimentally determined binding registers of nine gluten epitopes reveal positioning of proline residues in positions P1, P3, P6, and P8 but never in positions P2, P4, P7, and P9. Glutamate residues formed by tissue transglutaminase-mediated deamidation are found in position P1, P4, P6, P7, or P9, but only deamidations in positions P4 and P6, and rarely in P7, seem to be crucial for T cell recognition. The majority of these nine epitopes are recognized by celiac lesion T cells when presented by the related but nonassociated DQ2.2 (DQA1*0201, DQB1*02) molecule. Interestingly, the DQ2.2 presentation for most epitopes is less efficient than presentation by the DQ2.5 molecule, and this is particularly prominent for the alpha-gliadin epitopes. Contrary to previous findings, our data do not show selective presentation of DQ2.5 over DQ2.2 for gluten epitopes that carry proline residues at the P3 position.
Amino Acid Sequence↗
Studies of celiac disease. I. The apparent identical and specific nature of the duodenal and proximal jejunal lesion in celiac disease and idiopathic sprue.
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Celiac Disease↗
Studies of celiac disease. I. The apparent identical and specific nature of the duodenal and proximal jejunal lesion in celiac disease and idiopathic sprue.
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Adult↗
Occurrence of secondary cystathioninuria in children with inherited metabolic disorders, liver diseases, neoplasms, cystic fibrosis and celiac disease.
Secondary cystathioninuria was found in two of 46 children suffering from tumors, leukemia, liver disease, inherited metabolic disorders, cystic fibrosis and celiac disease. Of these two patients, one had congenital biliary atresia and the other cytomegalovirus infection. Seven further children had only moderately elevated excretion of cystathionine. It is suggested that secondary cystathioninuria is uncommon in the disease investigated.
Adolescent↗
Increased risk of surgery in undiagnosed celiac disease.
The diagnosis of celiac disease patients may be delayed by misdiagnosis. Our aim was to evaluate in celiac patients the prevalence of surgery before diagnosis. Two hundred forty-four adult celiac patients and 232 controls were retrospectively investigated for surgery before diagnosis of celiac disease. The prevalence of surgery was increased in celiac patients versus controls (P = 0.001). Frequency of appendectomy (P = 0.0001), tonsillectomy (P = 0.009), and hernia repair (P = 0.05) were increased in celiac patients versus controls. Appendectomy was related to anemia (P = 0.006) and abdominal pain (P = 0.005); tonsillectomy was related to diarrhea (P = 0.02) and weight loss (P = 0,04). Appendectomy was elective in 73% of celiac patients and in 46% of controls. Cosmetic surgery was increased in celiac patients versus controls (P = 0.058). In conclusions, surgery before celiac disease diagnosis is increased in celiac patients compared to controls, as a result of doctors' misdiagnosis and/or poor health status, which increases the demand for medical intervention. The frequency of cosmetic surgery in celiac patients may berelated to impaired psychological profile of patients.
Adult↗
Celiac disease and childhood cancer.
Celiac disease is an autoimmune enteropathy developing in genetically predisposed individuals after mucosal contact with gluten, secondary to unknown triggering factors. An increased rate of malignancies in adults with celiac disease has been confirmed in several studies, but there is considerable evidence that a gluten-free diet protects against the development of cancer. The association between celiac disease and childhood cancer is not clear. Few cases are reported in the literature. Here, we report the cases referred to our pediatric oncology unit with a review of the literature.
Autoimmune Diseases↗
Celiac disease--a worldwide problem.
Celiac disease and dermatitis herpetiformis are caused by the alcohol soluble fractions of wheat, barley, and rye. Reliable serological tests are available for both mass and risk group screening and recent epidemiological studies on celiac disease suggest that the prevalence varies between 1:100-300 in different continents. The clinical manifestations of the disease has changed in the West and the classical symptomatic cases represent only approximately 1/7th of all diagnosed cases. Symptoms such as, anemia, short stature, dental enamel defect or osteoporosis can be the only manifestations of the atypical disease. There is an increased prevalence of celiac disease in patients with autoimmune diseases. Recent data suggest that there is a correlation between the prevalence of autoimmune diseases and the number of years that an individual consumes gluten-containing foods. Genetic studies revealed a high prevalence of certain HLA antigens in celiac patients, however, there is likelihood that non-HIA genes are also important in the pathomechanism. An interesting new development is the recognition of tissue transglutaminase (tTG), an enzyme that probably forms an autoantigen with gluten. It is generally accepted that antibodies to tTG are identical to the previously described antiendomysium antibodies. Whether or not tTG is responsible for the initiation of an immunoreaction against prolamines or just exacerbates the immune response is a subject of further investigations.
Age of Onset↗
[Current concepts on celiac disease physiopathology].
Celiac sprue (CS) is defined as a chronic small bowel malabsorption disorder caused by ingestion of gluten, affecting those genetically predisposed individuals. It is characterized by intestinal villi atrophy, increased number of intraepithelial lymphocytes and extense inflammatory infiltrate in the intestinal lamina propria. The role of gluten as responsible for the intestinal damage seen in CS patients is clear, however, the physiopathological mechanisms involved are still unknown. Several factors and theories have been proposed: 1) Genetic predisposition, based on the association to mendelian factors as well to the presence of particular major histocompatibility complex (MHC) haplotypes in CS patients; 2) Immunological factors, that consider the derangements that occur in the immune response of CS patients, and 3) Gliadin partial deamination by the tissular transglutaminase (tTG). In an effort to explain all these complex mechanisms, recently, all these theories have been unified, yielding one complex physiopathogenic mechanism that we tray to explain in the present review.
Celiac Disease↗
[Serum antibodies in celiac disease].
Accurate diagnosis of celiac disease is important because patients are advised to adhere to a strict gluten-free diet for life. This management is critical to avoid disease complications such as malignancies. In this review the new terminology for the disease clinical features (active, silent, latent and potential celiac disease) and the disease association with other conditions are commented. The value and efficacy of the assessment of serum antigliadin antibodies and of antireticulin, antiendomysial and tissue transglutaminase autoantibodies in the diagnosis and follow-up of the celiac disease are particularly evaluated.
Antibodies↗