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At least 415 records · Page 23Linked to original sources
PubMed2000

Current concepts of celiac disease pathogenesis.

Our knowledge of celiac disease pathogenesis has recently made rapid progress. The disorder is now considered the result of a complex interplay of intrinsic (genetic) and variable extrinsic (environmental) factors that explain the wide spectrum of clinical manifestations ranging from asymptomatic to severe malabsorption. Gluten peptides are efficiently presented by celiac disease-specific HLA-DQ2- and HLA-DQ8-positive antigen-presenting cells, and thus drive the immune response, predominantly in the connective tissue of the lamina propria. Tissue transglutaminase, which has been identified as the highly specific endomysial autoantigen, is released from cells during inflammation. It may potentiate antigen presentation by HLA-DQ2 and HLA-DQ8 by deamidating or cross-linking gluten peptides. The result is lamina propria T-cell activation and mucosal transformation by activated intestinal fibroblasts. In the future, manipulation of the gut-associated lymphoid tissue may allow reduced sensitivity or even generate oral tolerance to gluten. Long-standing untreated celiac disease, even if clinically silent, predisposes for other autoimmune diseases. Therefore, population screening for immunoglobulin A antibodies to tissue transglutaminase seems justified.

Autoantigens↗
PubMed1994

High risk genotypes for celiac disease.

It is known that celiac disease is strongly associated with an HLA class II component and that most patients carry the dimer DQA1*0501, DQB1*0201. We show in this study that the risk for a carrier of this heterodimer is independent from the number of possible heterodimers, from whether DQA1*0501 and DQB1*0201 are in cis or trans position and from the number of DQA1*0501 (one or two) but strongly depends on the number of DQB1*0201. In the Tunisian population we studied, the risk of developing celiac disease is estimated to be 6.8 times greater for those having a double dose of DQB1*0201 than for other dimer carriers. We replicated this result in published data of four other populations (Italy, Czekoslovakia, United Kingdom, Norway).

Adolescent↗
PubMed2003

Causes of death in patients with celiac disease in a population-based Swedish cohort.

BACKGROUND: Patients with celiac disease have an increased risk of death from gastrointestinal malignancies and lymphomas, but little is known about mortality from other causes and few studies have assessed long-term outcomes. METHODS: Nationwide data on 10 032 Swedish patients hospitalized from January 1, 1964, through December 31, 1993, with celiac disease and surviving at least 12 months were linked with the national mortality register. Mortality risks were computed as standardized mortality ratios (SMRs), comparing mortality rates of patients with celiac disease with rates in the general Swedish population. RESULTS: A total of 828 patients with celiac disease died during the follow-up period (1965-1994). For all causes of death combined, mortality risks were significantly elevated: 2.0-fold (95% confidence interval [CI], 1.8-2.1) among all patients with celiac disease and 1.4-fold (95% CI, 1.2-1.6) among patients with celiac disease with no other discharge diagnoses at initial hospitalization. The overall SMR did not differ by sex or calendar year of initial hospitalization, whereas mortality risk in patients hospitalized with celiac disease before the age of 2 years was significantly lower by 60% (95% CI, 0.2-0.8) compared with the same age group of the general population. Mortality risks were elevated for a wide array of diseases, including non-Hodgkin lymphoma (SMR, 11.4), cancer of the small intestine (SMR, 17.3), autoimmune diseases (including rheumatoid arthritis [SMR, 7.3] and diffuse diseases of connective tissue [SMR, 17.0]), allergic disorders (such as asthma [SMR, 2.8]), inflammatory bowel diseases (including ulcerative colitis and Crohn disease [SMR, 70.9]), diabetes mellitus (SMR, 3.0), disorders of immune deficiency (SMR, 20.9), tuberculosis (SMR, 5.9), pneumonia (SMR, 2.9), and nephritis (SMR, 5.4). CONCLUSION: The elevated mortality risk for all causes of death combined reflected, for the most part, disorders characterized by immune dysfunction.

Adolescent↗
PubMed1980

Celiac disease and malignancy.

Fifty-five patients with celiac disease and coexistent malignant disease (27 lymphoma, 28 other malignancies) are described. The important clinical features at presentation of lymphoma were weight loss, abdominal pain, diarrhea, profound weakness and fever, associated with anemia, raised ESR, hypoalbuminemia and steatorrhea. There were no specific features to enable earlier diagnosis. Radiology was unhelpful and in no case were malignant cells seen in the jejunal biopsy. Four of the lymphomas were Hodgkin's disease, none of which involved the bowel; the remainder were reticulum cell sarcoma, 17 of which involved the bowel. Definitive diagnosis prior to death was made in only 18 patients, of whom 16 survived from 2 to 226 days (mean, 76 days). Of the remaining two patients, one is still alive, while the other died 26 years after the original diagnosis of Hodgkin's disease. The possibility of lymphoma should be considered in those who present with celiac disease in middle life and in those who deteriorate for no apparent reason after a period of stability on a gluten-free diet. The index of suspicion for lymphoma in celiac disease should be high and early laparotomy be considered in patients with unexplained deterioration. Twenty-eight patients with 29 carcinomas and 3 other tumors are also described. The presentations of these malignancies were no different from their presentations in non-celiac patients, and their development did not provoke a relapse of celiac disease. Considering the whole series of 55 patients, there was little evidence for the view that malignancy itself was the cause of the flat jejunal mucosal appearances seen in these patients.

Adult↗
PubMed2005

Small-fiber neuropathy/neuronopathy associated with celiac disease: skin biopsy findings.

BACKGROUND: Celiac disease (CD) is increasingly recognized in North America and is associated with a peripheral neuropathy. OBJECTIVE: To report the clinical characteristics and skin biopsy results in patients with CD and small-fiber neuropathy symptoms. DESIGN: Case series. SETTING: Academic peripheral neuropathy clinic. PATIENTS: Eight patients with CD and neuropathy symptoms. Intervention Three-millimeter punch biopsy using the panaxonal marker protein gene product 9.5 to assess epidermal nerve fiber (ENF) density and a gluten-free diet. MAIN OUTCOME MEASURE: Clinical data and ENF density. RESULTS: All patients had asymmetric numbness and paresthesias. Three had more prominent involvement of hands than feet, and 3 had facial numbness. Celiac disease was diagnosed in 5 after their neuropathy began. The following serum antibody levels were elevated: tissue transglutaminase (n = 6), IgA gliadin (n = 4), and IgG gliadin (n = 7). Results of nerve conduction studies were normal in 7 patients. One patient had mildly reduced sural amplitudes. The ENF density was reduced in 5 patients. The ENF density was at the low limit of the normal range in 3 additional patients, 2 of whom had morphologic changes in axons. Three patients had decreased ENF density at the thigh or forearm, which was more severe than at the distal leg, compatible with a non-length-dependent process. Four reported improvement with a gluten-free diet. One had no improvement after 4 months. Symptoms developed in 2 while receiving a gluten-free diet. CONCLUSIONS: Patients with CD may have a neuropathy involving small fibers, demonstrated by results of skin biopsy. The pattern of symptoms, with frequent facial involvement and a non-length-dependent pattern on skin biopsy findings, suggests a sensory ganglionopathy or an immune-mediated neuropathy. Improvement of symptoms in some patients after initiating a gluten-free diet warrants further study.

Celiac Disease↗
PubMed2001

Nodular regenerative hyperplasia of the liver in a patient with celiac disease.

We present the case of dual adult celiac disease and liver disease with portal hypertension (esophageal varices); a percutaneous liver biopsy was compatible with nonspecific reactive hepatitis. Clinically, celiac disease was characterised by poor response to a gluten-free diet, with the development of a biochemical cholestasis and marked malnutrition. Our patient died of cerebral hemorrhage, at the age of 50 years, without associated risk factors. The necropsy demonstrated the existence of a nodular regenerative hyperplasia of the liver, splenic atrophy, gelatinous transformation of the bone marrow, and lymphocytic colitis. We discuss the different types of liver disorders associated with celiac disease and the possible relation between nodular regenerative hyperplasia and celiac disease, based on immunologic mechanisms.

Autopsy↗
PubMed1994

Associations between alleles of the major histocompatibility complex-encoded ABC transporter gene TAP2, HLA class II alleles, and celiac disease susceptibility.

The study of celiac disease among Southern European populations has confirmed the hypothesis from Northern Europe that a close association exists between disease susceptibility and a combination of polymorphic alleles at the HLA-DQ loci (DQA1*0501-DQB1*0201) arranged either in a cis- or a trans-configuration. Attempts to identify additional genetic influences have been inconclusive, although many studies have raised possibilities of further major histocompatibility complex (MHC)-linked susceptibility genes. This study examines the disease associations of polymorphisms of a recently discovered gene located close to the DQ loci, TAP2, whose products are thought to be involved in the transport of antigen peptides across the endoplasmic reticulum for binding to HLA class I molecules. Like the products of the DQ loci, the product of TAP2 forms part of a heterodimeric molecule with products of a second genetic locus, TAP1, which is located centromerically to TAP2. The populations studied were central Italians from Rome and Ashkenazi Jews from Rehovot, Israel. HLA studies demonstrate that the Roman celiac group has a high proportion of people with DR3-negative celiac disease in whom the combination DR5/7 is frequently found. In Israel, 20% of celiac patients lack the DQ susceptibility markers but are DR4 positive. The polymorphic substitutions of TAP2 studied encode amino acid changes in the trans-membrane region (position 379) and the ATP-binding cassette region (positions 565 and 665) of the protein. No TAP2-specific disease associations were found in any HLA subgroup.(ABSTRACT TRUNCATED AT 250 WORDS)

ATP Binding Cassette Transporter, Subfamily B, Mem↗
PubMed1999

Idiopathic cerebellar ataxia associated with celiac disease: lack of distinctive neurological features.

OBJECTIVES: To determine the occurrence of celiac disease in a population of ataxic patients without definite diagnosis and to characterise distinctive features which may help to differentiate cerebellar ataxia with and without celiac disease. METHODS: Twenty four ataxic patients without definite diagnosis (group A) and 23 ataxic patients with definite diagnosis (group B) were screened for antigliadin (AGAs) and antiendomysium antibodies (EMAs). Patients with a positive AGA or EMA test underwent endoscopic biopsy of the duodenal mucosa. RESULTS: There was an increased prevalence of celiac disease in group A (3/24) compared with group B (0/23). None of the celiac patients presented gastrointestinal symptoms or malabsorption signs. None of the ataxic patients with celiac disease had early onset ataxia. CONCLUSIONS: Celiac disease is associated with ataxic syndromes without definite diagnosis, suggesting that it plays a part in the pathogenesis of some ataxic syndromes. The absence of distinctive neurological features in ataxic patients with celiac disease suggests that a search should be made for celiac disease markers in all ataxic patients without definite diagnosis.

Adult↗
PubMed1998

Chronic progressive leukoencephalopathy in adult celiac disease.

Progressive leukoencephalopathy developed in a patient with adult celiac disease. Neurologic abnormalities appeared 4 years after the gastrointestinal manifestations despite a gluten-free diet and replacement of vitamins. Brain MRI showed marked confluent white matter abnormalities, and stereotactic brain biopsy revealed chronic leukoencephalopathy. Treatment with I.V. steroids and immunoglobulins did not stop disease progression. Celiac disease should be considered in the differential diagnosis of the leukoencephalopathies.

Biopsy↗
PubMed2000

[Clinical relevance of immunoglobulin A deficiency in celiac disease].

BACKGROUND: To determine the prevalence of the IgA deficiency in patients with celiac disease and its influence on the presentation and evolution of celiac disease. PATIENTS AND METHOD: 47 patients with clinical and histological diagnosis of celiac disease were included. Seric immunoglobulin levels were determined in all patients. RESULTS: Five patients presented a low level of IgA (IgA-deficient group) and 42 patients presented normal levels (non-IgA-deficient group). The age and the relation male/female were similar between both groups. Presentation, clinical manifestations, analytic data of intestinal malabsorption, and the radiologic pattern were also similar between both groups of patients. There were bronchiectasies in two out of 5 IgA-deficient patients and in only one out of 42 non-IgA- deficient patients (p < 0.05). None of the IgA-deficient group showed complications due to the celiac disease, meanwhile 3 non-IgA deficient patients developed intestinal lymphoma (p = NS). CONCLUSIONS: The IgA deficiency is frequently associated to celiac disease. This association does not seem to influence in a significant way the presentation or the evolution of celiac disease.

Adult↗
PubMed2000

Body composition and dietary intakes in adult celiac disease patients consuming a strict gluten-free diet.

BACKGROUND: Celiac disease responds to dietary gluten withdrawal, but data on the long-term effects of gluten-free diets are discordant. OBJECTIVE: Our aim was to evaluate the nutritional status and body composition of adult celiac disease patients consuming a gluten-free diet who were in clinical, biochemical, and histologic remission. DESIGN: We studied 71 patients (51 women and 20 men; mean age: 27 y; range: 17-58 y) and 142 healthy control subjects matched by sex and age. The subjects' height, weight, body mass index, fat and lean mass, and bone mineral content (evaluated by dual-energy X-ray absorptiometry) were measured; a 3-d dietary questionnaire was administered; and total daily energy, fat, carbohydrate, and protein intakes were calculated. RESULTS: The weight, height, and body mass index of male celiac disease patients and the weight and body mass index of female celiac disease patients were significantly lower than the corresponding measurements in control subjects. The fat and lean mass of both male and female patients was significantly different from that of control subjects; however, bone mineral content was significantly lower only in females in whom celiac disease was diagnosed in adulthood. Total energy intake was lower in the patients than in the control subjects (9686 +/- 1569 and 11297 +/- 1318 kJ/d in males and 6736 +/- 1318 and 7740 +/- 1715 kJ/d in females), and the diet of the patients was unbalanced, with a higher percentage of energy as fat and a lower percentage of energy as carbohydrates. CONCLUSIONS: Although strictly compliant with their gluten-free diet and in complete remission, patients with celiac disease showed differences in body composition and dietary intakes compared with control subjects. Strict follow-up and dietary advice in terms of the choice and composition of foods seem necessary to prevent malnutrition.

Absorptiometry, Photon↗
PubMed2000

Transglutaminase antibodies in children with a genetic risk for celiac disease.

OBJECTIVES: The transglutaminase (TG) antibody test is accurate in identifying celiac disease in symptomatic children. We sought to determine the positive predictive value of this test in asymptomatic children at genetic risk for celiac disease. STUDY DESIGN: Asymptomatic children with a genetic risk for celiac disease were studied to investigate the relationships between TG antibody titer, small bowel histology, growth, and clinical features. Small bowel biopsy histology was graded by using the system of Marsh. RESULTS: Of 30 children with a positive TG antibody test result, 21 (70%) had definite (Marsh score 2 or 3) and 4 (13%) had possible (Marsh score 1) biopsy evidence of celiac disease. TG antibody titer correlated with Marsh score (r = 0.569, P <.01). There was an inverse correlation between Marsh score and height z score (r = -0.361, P =. 05). CONCLUSIONS: In this group of asymptomatic children screened because of a genetic risk, TG antibodies have a positive predictive value of 70% to 83% for biopsy evidence of celiac disease and may identify children before clinical features of celiac disease develop.

Adolescent↗
PubMed2002

Celiac disease in patients with severe liver disease: gluten-free diet may reverse hepatic failure.

BACKGROUND & AIMS: Mild liver abnormalities are common in patients with celiac disease and usually resolve with a gluten-free diet. We investigated the occurrence of celiac disease in patients with severe liver failure. METHODS: Four patients with untreated celiac disease and severe liver disease are described. Further, the occurrence of celiac disease was studied in 185 adults with previous liver transplantation using serum immunoglobulin A endomysial and tissue transglutaminase antibodies in screening. RESULTS: Of the 4 patients with severe liver disease and celiac disease, 1 had congenital liver fibrosis, 1 had massive hepatic steatosis, and 2 had progressive hepatitis without apparent origin. Three were even remitted for consideration of liver transplantation. Hepatic dysfunction reversed in all cases when a gluten-free diet was adopted. In the transplantation group, 8 patients (4.3%) had celiac disease. Six cases were detected before the operation: 3 had primary biliary cirrhosis, 1 had autoimmune hepatitis, 1 had primary sclerosing cholangitis, and 1 had congenital liver fibrosis. Only 1 patient had maintained a long-term strict gluten-free diet. Screening found 2 cases of celiac disease, 1 with autoimmune hepatitis and 1 with secondary sclerosing cholangitis. CONCLUSIONS: The possible presence of celiac disease should be investigated in patients with severe liver disease. Dietary treatment may prevent progression to hepatic failure, even in cases in which liver transplantation is considered.

Adolescent↗
PubMedDate not supplied

[Celiac disease: clinical characteristics and methods used in the diagnosis of patients registered at the Brazilian Celiac Association]

OBJECTIVE: To evaluate the clinical characteristics and methods used in the diagnosis of patients registered at the Brazilian Celiac Association (BCA). METHODS: A questionnaire about clinical characteristics and diagnostic methods in celiac disease was mailed to 584 members of the BCA. RESULTS: We received 292 responses for 584 questionnaires mailed (49.5%). The clinical characteristics of celiac disease in the sample we analyzed showed that the most frequent type was the classical mode (88.9%), while the atypical mode was present in only 11.1% of patients at the time of diagnosis. Increased incidence of both late diagnosed classical mode (44.5% to 64.2%; P=.004) and atypical mode (5.2% to 16.8%; P=.005) has been observed in the last 5 years. Duration of symptoms before diagnosis was greater than 1 year in 75% patients with atypical manifestation. Intestinal biopsy was not performed in 19% of the cases at the time of diagnosis. It was observed that in the last 5 years intestinal biopsy was not performed for a larger number of patients (24.4%) than in the previous period (only 11.1%) (P=.007). CONCLUSIONS: Classical mode is still the most frequent clinical manifestation of celiac disease. The late diagnosed classical mode is predominant, but our findings show an increase in the proportion of the atypical mode. Although characterization of subtotal or total villous atrophy of the intestinal mucosa is of paramount importance to the diagnosis of celiac disease, 19% of the patients diagnosed for celiac disease did not undergo intestinal biopsy at the time of diagnosis, most notably in the last 5 years.

Journal Article↗
PubMed2004

Increasing age at diagnosis of celiac disease in Malta.

OBJECTIVE: Recent studies have shown that celiac disease is being diagnosed at a progressively later age. This paper analyses trends in age at diagnosis in a closed island population. METHODS: Patient case notes of all known patients with celiac disease were retrieved and demographic information, mode of presentation including symptomatology and diagnostic criteria were obtained. Over the period 1985 to 2000, information was available on 42 patients with celiac disease. All were aged 14 years. RESULT: The mean age at diagnosis showed an increasing age, with a constant disease incidence. A highly significant positive correlation was found for age at diagnosis with time (rho=0.4, p=0.009). CONCLUSION: It is thought that breast feeding, with later introduction of gluten containing products in the diet, leads to a later presentation of celiac disease. In Malta, over the period under study, the breast feeding rate rose from 20% to 60%. Our findings support the hypothesis that breast feeding offers a degree of protection against the early development of celiac disease, without actually reducing the incidence of the disease.

Age of Onset↗
PubMed1995

Celiac disease and alopecia areata: report of a new association.

Celiac disease is frequently associated with other autoimmune disorders but has never been reported in association with alopecia areata. In a routine clinical practice, 3 patients with such an association were observed. In one of the patients, celiac disease was diagnosed after the occurrence of malabsorption symptoms. In the youngest patient, a 14-year-old boy, gluten-free diet resulted in complete regrowth of scalp and body hair. A prospective screening program for celiac disease using antigliadin and antiendomysial antibodies was therefore set up in 256 consecutive outpatients with alopecia areata. Three patients, all completely asymptomatic for intestinal diseases, were found to be positive and underwent biopsy. Histological analysis showed a flat intestinal mucosa consistent with the diagnosis of celiac disease. The results show that alopecia areata may constitute the only clinical manifestation of celiac disease and that the association between these two conditions is a real one because the observed frequency of association is much greater than can be expected by chance. It is suggested that antigliadin and antiendomysial antibodies should be included in the work-up of patients with alopecia areata.

Adolescent↗
PubMedDate not supplied

T cell lymphoma of the thyroid gland in celiac disease.

Previous studies have reported the association between celiac disease and T cell lymphoma of the intestine as well as hepatosplenic lymphoma, a specialized peripheral type of T cell lymphoma. In this report, a 66-year-old woman with dermatitis herpetiformis and biopsy-defined celiac disease developed a thyroid mass that proved to be a T cell lymphoma. A T cell lymphoma in the setting of celiac disease appears to be unique. The thyroid gland, due to its shared embryological developmental links with the gastrointestinal tract, is possibly another site of extranodal lymphoma linked to celiac disease.

Aged↗