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PubMed1999

Celiac disease in relation to immunologic serum markers, trace elements, and HLA-DR and DQ antigens in Swedish children with Down syndrome.

BACKGROUND: An association between Down syndrome and celiac disease has been reported. This study was conducted to determine the association between childhood celiac disease and Down syndrome in the county of Uppsala, Sweden. METHODS: All 76 children with Down syndrome (1-18 years) were screened for the occurrence of anti-gliadin antibodies (AGA) and anti-endomysium antibodies (EMA). Twelve children with suspected celiac disease were investigated further. RESULTS: Increased levels of both IgA and IgG AGA were found in 26% of the children and of EMA in and 5 of 76. Celiac disease was diagnosed in at least three of the children (3.9%; 95% confidence interval 0%-8.3%), and it could have been present in as many as eight. Three of the five EMA-positive children with suspected celiac disease had the HLA phenotype DR3, DQ2. CONCLUSIONS: The results show that determination of EMA is more useful as a screening test for celiac disease and for follow-up than is AGA in children with Down syndrome. The present study also confirms that celiac disease is overrepresented among Swedish children with Down syndrome and that celiac disease should be considered in all persons with Down syndrome.

Adolescent↗
PubMed2005

Should all children be screened for celiac disease?

To address the issue of screening children for celiac disease, current evidence has been summarized and placed within the context of 8 established criteria for childhood screening. Screening all children for celiac disease is not recommended at this time. Areas with gaps in knowledge are identified as areas in need of further study. These include the timing of screening, defining the natural history of screening-identified celiac disease, developing tools to predict disease onset and disease remission, and the risks of screening.

Age Factors↗
PubMed2000

Adult celiac disease and hypertransaminasemia.

OBJECTIVE: to determine the incidence of hypertransaminasemia in adult patients with celiac disease with or without relevant chronic liver disease, and to evaluate the response after a gluten-free diet. PATIENTS AND METHODS: retrospective study of 20 cases of adult celiac disease (> 14 years old at diagnosis). Patients were included in the study if they fulfilled the revised EPSGAN criteria. If laboratory tests of liver function revealed alterations, hepatitis B and C viral serology, thyroid hormones, and use of alcohol and drugs were investigated, and liver ultrasound scans were done. Liver biopsy and endoscopic retrograde cholangiopancreatography were done only in patients for whom these studies were considered necessary. RESULTS: ten patients had hypertransaminasemia (50%), ascribed to benzodiazepine use in 1 patient, chronic HCV hepatitis in 1, and celiac disease in 8. In all of these last patients except 1 (benzodiazepine use), laboratory values returned to normal after 4-10 months on a gluten-free diet. CONCLUSIONS: celiac disease was frequently associated with hypertransaminasemia. In most patients transaminase levels returned to normal within 1 year after dietary gluten intake was restricted. If alterations in laboratory values persist, other causes that may be related (e.g., autoimmunity or tumors) or unrelated to celiac disease (e.g., virus) must be ruled out.

Adult↗
PubMed2003

Risk of malignancy in patients with celiac disease.

PURPOSE: Studies from Europe have demonstrated an increased risk of malignancy, especially non-Hodgkin's lymphoma, in patients with celiac disease. However, there are no data on the risk for similar patients in the United States. Our aim was to estimate the risk of malignancy in a cohort of patients with celiac disease compared with the general U.S. population and to determine if a gluten-free diet is protective. METHODS: Patients with celiac disease seen between July 1981 and January 2000 at a referral center were included. Standardized morbidity ratios (SMRs) (ratio of observed to expected) and corresponding 95% confidence intervals (CI) were calculated, using data from the National Cancer Institute's Surveillance, Epidemiology, and End Results Program. RESULTS: Forty-three (11%) of 381 celiac disease patients had a diagnosis of cancer; 9 were after the diagnosis of celiac disease, 7 were simultaneous (during same month or admission), and 27 were before the diagnosis. The standardized morbidity ratio for all cancers combined was 1.5 (95% CI: 0.3 to 7.5), with significantly increased values for small bowel cancer (SMR = 34; 95% CI: 24 to 42), esophageal cancer (SMR = 12; 95% CI: 6.5 to 21), non-Hodgkin's lymphoma (SMR = 9.1; 95% CI: 4.7 to 13), and melanoma (SMR = 5.0; 95% CI: 2.1 to 12). Following the diagnosis of celiac disease, patients were at increased risk of non-Hodgkin's lymphoma only (SMR = 6.2; 95% CI: 2.9 to 14), despite adherence to a gluten-free diet. The non-Hodgkin's lymphoma included both T-cell and B-cell types and occurred in both gastrointestinal (n = 5) and extraintestinal sites (n = 4). CONCLUSION: In this cohort of patients with celiac disease, we observed increased risks of small intestinal adenocarcinoma, esophageal cancer, melanoma, and non-Hodgkin's lymphoma. The risk of non-Hodgkin's lymphoma persisted despite a gluten-free diet.

Adult↗
PubMed1994

IgA antiendomysium antibodies have a high positive predictive value for celiac disease in asymptomatic patients.

Many attempts have been made to find screening tests for celiac disease to reduce the need for biopsy, or to achieve better selection criteria before intestinal biopsy. We have recently analyzed apparently healthy blood donors for antigliadin antibodies (AGA) to select subjects for further gastrointestinal investigation. A prevalence of gluten enteropathy of at least 1/256 was found in this population. The positive predictive value (+PV), however, was only 20%. In the present study we have analyzed IgA antiendomysium antibodies (IgA-EmA) to estimate the sensitivity and specificity of the test, and determine whether or not the +PV of the assay increases when screening for adult celiac disease in an asymptomatic population. We found that asymptomatic persons with celiac disease may have IgA-EmA. We found a 100% specificity of IgA-EmA in the tested population of blood donors, whereas the sensitivity was about the same as that of IgA-AGA. This result of a +PV of 100% indicates that a positive IgA-EmA could replace biopsy in diagnosing celiac disease. However, further extended studies are needed to determine whether this is applicable in other populations. To screen patients for celiac disease, we recommend the easy and cheap IgA-AGA assay as a preliminary test and the IgA-EmA to verify the diagnosis and avoid unnecessary biopsies.

Adult↗
PubMed2002

Enteroclysis in adult celiac disease: diagnostic value of specific radiographic features.

The purpose of this study was to compare the diagnostic accuracy of various radiographic findings at enteroclysis in adult patients with untreated celiac disease. Twenty-seven adult patients underwent enteroclysis because of unspecific intestinal symptoms before definitive biopsy proof of celiac disease. Enteroclysis of 123 subjects with similar clinical presentation, including abdominal pain, diarrhea, occult intestinal bleeding, and weight loss, who had a definitive diagnosis other than celiac disease, served as controls. The radiographic features previously described in the literature as indicative of adult celiac disease (i.e., fold thickening, decrease of jejunal folds, increase of ileal folds, small bowel dilatation, flocculation) were evaluated in blinded fashion in all studies and the subjective likelihood of diagnosis of celiac disease was assessed. Assessing every finding separately, each feature proved to have a high specificity (78-100%) but low sensitivity (19-59%) for celiac disease. Reversal of jejunoileal fold pattern was the single best feature (specificity 100%, 95% CI 97-100%; sensitivity 59%, 95% CI 40-78%); however, combination of criteria enables establishment of the diagnosis of celiac disease quite accurately (specificity 100%, 95% CI 98-100%; sensitivity 78%, 95% CI 58-91%). Reversal of jejunoileal fold pattern as a single finding as well as combination at least three of the following features, i.e., fold thickening, decrease of jejunal folds ("colonization"), increase of ileal folds ("jejunization"), dilatation, and flocculation, make enteroclysis an accurate tool for diagnosis of celiac disease in adult patients with suspected intestinal disease.

Adult↗
PubMed2002

A controlled, prospective screening study of celiac disease presenting as iron deficiency anemia.

BACKGROUND: anemia is the most frequent presenting feature of celiac disease in adults using endomysial antibody (EmA) screening. Endomysial antibody screening of anemia may allow detection of celiac disease at an earlier stage of investigation and after a shorter duration of symptoms. The characteristics of celiac patients identified by screening require further study. GOALS: a goal of this study was to evaluate the prevalence of celiac disease in adult patients with iron deficiency anemia compared with a nonanemic control population using immunoglobulin A (IgA) EmA screening. We also studied the positive predictive value (PPV) of the EmA assay and correlated the severity of histologic abnormalities in distal duodenal biopsy samples with EmA and tissue transglutaminase (tTG) antibody titer. STUDY: four hundred eighty-four patients with microcytic, hypochromic anemia underwent IgA EmA assay. Four hundred ninety-eight nonanemic age- and sex-matched patients from the same source comprised the control group. Patients with positive serology results were invited for endoscopic duodenal biopsies. RESULTS: one in 44 anemic patients was diagnosed with histologically confirmed celiac disease compared with one in 498 nonanemic patients ( < 0.01). Fifty percent of women were premenopausal, and 25% of patients were older than 65 years. The PPV for EmA assay varied between 73% and 93% for anemic patients and improved at higher antibody titer, with all false-positive results occurring at the lowest titers. CONCLUSIONS: screening for celiac disease using IgA EmA assay is effective in anemic patients, including premenopausal women and patients older than 65 years, and it can be recommended in clinical practice.

Adult↗
PubMed2004

Prevalence of occult celiac disease in patients with iron-deficiency anemia: a prospective study.

BACKGROUND: Occult celiac disease has been reported in 0 to 6% of adults presenting with iron-deficiency anemia. Most prior studies have been retrospective or screened only a selected population of patients with small bowel biopsies. To more accurately define the true prevalence of this disorder in patients presenting with iron-deficiency anemia (with or without stool hemoccult positivity), we initiated this prospective study. METHODS: Esophagogastroduodenoscopy with small bowel biopsies and colonoscopy were performed in all iron-deficiency anemia patients (including those with hemoccult-positive stools) referred to the gastroenterology service during a 2-year period (1998-2000). Inclusion criteria included iron-deficiency anemia as defined by a serum ferritin < 25 ng/ml and anemia with hemoglobin < 12 g/dl. Patients were excluded for documented prior erosive, ulcerative, or malignant disease of the gastrointestinal tract, previous gastrointestinal surgery, overt gastrointestinal bleeding within the past 3 months, or inability to access the duodenum for biopsy. All patients underwent upper endoscopy with more than two biopsies of the distal duodenum and colonoscopy. A serum immunoglobulin A antiendomysial antibody test was to be performed in those patients with a positive small bowel biopsy to confirm the diagnosis of celiac disease. RESULTS: One hundred five of 139 consecutive patients with iron-deficiency anemia met the inclusion criteria and were enrolled in the study. Fifty-seven men (mean age, 51.6 yr) and 48 women (mean age, 54.1 yr) constituted the study population. The demographics of this study population included 36 blacks, 38 Hispanics, and 22 whites. Nine patients were of mixed or unknown ethnic background. Forty-three and eight-tenths percent of the men and 37.5% of women had hemoccult-positive stools, accounting for a total of 40.9% of the study patients. Upper endoscopic findings included gastritis in 22.8%, gastric ulcers in 9.5%, duodenitis in 8.5%, esophagitis in 7.6%, Barrett's ulcer in 2.8%, duodenal ulcer in 2.8%, gastric polyp in 2.8%, and celiac disease in 2.8%. Colonoscopic findings included colon polyps in 21.9%, diverticula in 10.4%, and hemorrhoids in 16.1%. Multiple findings were found in 32.3% of patients, and there were no findings in 28.5% of patients. CONCLUSION: The prevalence of occult celiac disease in this prospective study of patients presenting with iron-deficiency anemia was 2.8%. A significant number of other gastrointestinal lesions amenable to therapy were also found on upper and lower endoscopy in these patients. Given the treatable nature of celiac disease, it should be screened for in patients with unexplained iron-deficiency anemia with or without hemoccult-positive stools.

Anemia, Iron-Deficiency↗
PubMed2001

Diagnostic approach to a patient with suspected celiac disease: a cost analysis.

Serological testing is an important tool in the diagnostic work-up of suspected celiac disease. Our aim was to apply a decision analysis model to compare the costs of serological testing versus small bowel biopsy in the diagnostic work-up of celiac disease. A cost-minimization approach was employed. A decision analysis model with three diagnostic arms was designed using Data Version 3.5: anti-gliadin antibody versus endomysial antibody versus small bowel biopsy. Response to gluten-free diet was considered diagnostic of celiac disease; lack of response prompted a small bowel biopsy to definitively exclude celiac disease. Baseline probabilities were varied using sensitivity analysis. Sensitivity analysis revealed that the endomysial antibody strategy was least costly, provided the prevalence of celiac disease was less than 42%; above this anti-gliadin antibody became the most economical option. In conclusion, initial screening with endomysial antibody is the least costly strategy for diagnosing celiac disease in a low risk population. Antigliadin antibody becomes the cheaper strategy for higher risk populations.

Biopsy↗
PubMed2000

[Transglutaminase antibody: usefulness in the diagnosis of celiac disease].

AIM: Until now IgA-EmA and IgA-AGA antibodies have been considered to be specific and sensitive markers of celiac disease. Anew antibody, the tissue transglutaminase (IgA-tTG), antibody has recently been identified, which is believed to be if not a better marker then a more practical one in screening for celiac disease, especially in large populations. To evaluate the usefulness of the IgA-tTG antibody in the diagnosis and screening of celiac disease and to determine the relationship between this antibody and other better known and over-used antibodies, antigliadin (IgA-AGA) and antiendomysial (IgA-EmA). PATIENTS AND METHODS: The study was performed in 115 children divided into three groups: 31 patients diagnosed with the celiac disease, according to the ESPGAN criteria; 21 patients with celiac disease following a gluten free diet, and 63 considered as control group. Two enzyme linked immunoabsorbent assays (ELISA) were used to detect AGA and tTG antibodies, respectively. EmA antibodies were determined by using an indirect immunofluorescence technique with commercial sections of distal monkey oesophagus as antigen. RESULTS: In the 115 patients taken as a whole, the tTG antibody showed 91% agreement with the EmA antibody and 85% agreement with the AGA antibody. In the celiac group, agreement was 84% and 61% respectively. Sensitivity of the tTG antibody was 83% compared with 94% for EmA and 74% for AGA. Specificity was similar in all three tests. CONCLUSIONS: The ELISA test for tTG correlates well with the traditional AGA and EmA tests and could be used as a new test for celiac disease. The procedure is simple and shows high specificity and sensitivity compared with classical EmA and AGA tests and does not involve subjective scoring. It is cheap and very well suited for large scale screening for celiac disease.

Adolescent↗
PubMed2005

[Usefulness of anti-transglutaminase antibodies in the diagnosis of celiac disease].

BACKGROUND: Anti-transglutaminase antibodies have been proposed as a useful tool in the diagnosis and follow-up of celiac disease. AIM: To compare anti-transglutaminase antibodies with the classical antibodies used to diagnose celiac disease. MATERIAL AND METHOD: Patients who underwent duodenal biopsy for suspected celiac disease were selected if they had the following serum antibody samples: antiendomysial IgA, anti-transglutaminase IgA, antigliadin IgG, and antigliadin IgA. A diagnostic value of each of these antibodies was established according to the final diagnosis, taking the duodenal biopsy as the reference. RESULTS: One hundred twenty-two patients with duodenal biopsy and serologic markers for celiac disease were selected. Thirty-six patients were children (< 14 years-old) and 86 were adults. A diagnosis of celiac disease was made in 41 patients (26 children and 15 adults). Of the 15 adults, only 2 (13%) presented typical malabsorption syndrome. The sensitivity and specificity of anti-transglutaminase antibodies was 100% and 98% respectively compared with values of 97% and 98% for antiendomysial IgA, 85% and 97% for antigliadin IgA, and 97% and 92% for antigliadin IgG antibodies. CONCLUSIONS: The diagnostic value of anti-transglutaminase antibodies is high in celiac disease. These antibodies may be useful as a screening test to select candidates for duodenal biopsy.

Adult↗
PubMed2001

[Hypertransaminasaemia and impaired liver function in a patient with oligosymptomatic celiac disease].

Hypertransaminasaemia and impaired liver function in a patient with oligosymptomatic celiac disease. We describe the case of a 45-year-old man who was referred for evaluation of elevated aminotransferases. One year before referral the patient developed an ischemic stroke followed by a subdural hematoma three months later. In our outpatient clinic the patient presented with a malabsorption syndrome including diminished vitamin-K-dependent clotting factors. Serologic testing was positive for IgA antigliadin antibodies and IgA antiendomysial antibodies. Celiac disease was confirmed by an upper endoscopy examination and biopsies obtained from the distal duodenum. Histological examination showed villous atrophy, crypt hyperplasia and an increase in intraepithelial lymphocyte count consistent with celiac disease. After initiation of a gluten-free diet the malabsorption syndrome as well as liver dysfunction improved. Serum aminotransferase levels normalized within 6 months. The clinical course demonstrates involvement of the liver in patients with celiac disease.

Alanine Transaminase↗
PubMed2005

[Celiac disease, cerebral venous thrombosis and protein S deficiency, a fortuitous association?].

INTRODUCTION: Celiac disease is a pathology which is rarely associated with thrombosis complications. Cerebral vascular thrombosis has never been described in patients with a celiac disease. OBSERVATION: We report an observation of a 21-year-old girl with a history of celiac disease who was hospitalized in the intensive care unit for convulsive status epilepticus secondary to a cerebral venous thrombosis. The etiologic investigation of this thrombo-embolic complication revealed protein S deficit. Our patient improved under symptomatic treatment. COMMENT: This original observation confirms that celiac disease can be associated with cerebral venous thrombosis.

Adult↗
PubMedDate not supplied

Clinical evolution of celiac disease in Italy 1982-2002.

GOALS: The aim of this work is to assess how the clinical features of celiac disease have changed in Italy after the widespread introduction of serologic tests in 1993. STUDY: Twenty Italian Clinical Centers collected information from 1982 until 2002 on 1968 patients older than 18 years diagnosed with celiac disease. RESULTS: The results show that the incidence of atypical and silent cases of celiac disease has increased after the wider availability of serological test, which has allowed earlier diagnosis and treatment. CONCLUSIONS: This paper provides a view on the evolution of the clinical features of celiac disease in Italy over 2 decades. The relevance of the analysis is supported by the fact that Italy is one of the countries with the highest incidence of celiac disease. These findings underline the importance of a timely diagnosis of celiac disease.

Adult↗
PubMed1997

Production of antibodies to gliadin by peripheral blood lymphocytes in children with celiac disease: the use of an enzyme-linked immunospot technique for screening and follow-up.

Problems in the diagnosis of celiac disease are that a long time is needed between challenge with gluten and the appearance of the typical diagnostic morphologic signs in gut mucosa. Furthermore, local immunity to gliadin is only slowly and often incompletely mirrored by serum IgA anti-gliadin antibody (AGA) levels. It is known that a local IgA-associated immune response in the gut may be better and more quickly mirrored by an increase of circulating IgA-producing cells against the immunogen than by IgA serum antibodies. We have therefore used an enzyme-linked immunospot (ELISPOT) assay to enumerate IgA AGA spot-forming cells (SFC) in peripheral blood in 82 children with suspected celiac disease or with other gastrointestinal symptoms. The numbers of IgA AGA SFC/10(6) mononuclear cells were markedly increased in 17 patients with untreated (and later biopsy-verified) celiac disease compared with healthy children, children with nonceliac disease, and patients treated for celiac disease (p < 0.0001). In 20 children with celiac disease the numbers of IgA AGA SFC increased rapidly (p < 0.0001) after gluten challenge. As early as 2 wk after gluten challenge, 15/20 of these patients had abnormal levels of IgA AGA SFC, 6/20 patients had increased levels of serum IgA AGA, and 7/20 had IgA anti-endomysium antibodies. Our results indicate that analysis of IgA AGA production in peripheral blood cells may in further clinical studies provide a sensitive method for the diagnosis of celiac disease after a short time of gluten challenge.

Adolescent↗
PubMed1989

Adult celiac disease and its complications.

Classic radiographic findings described in adult celiac disease--lumen dilatation, flocculation of barium, hypersecretion, thickening of folds--are nonspecific or are secretion related artifacts. The small bowel enema technique and CT make it possible to demonstrate specific diagnostic features of the disease and its complications. This substantially modified approach to the diagnosis of adult celiac disease is presented together with correlative pathology. Examples of the complications of celiac disease--ulcerative jejunoileitis, lymphoma, hyposplenism, carcinoma, and the cavitary lymph node syndrome--are illustrated.

Adult↗
PubMed1979

Frequency of HLA-B8 in Israeli children with celiac disease.

The frequency of HLA-B8 in 18 Israeli children with celiac disease was examined and compared with that in 27 nonceliac children hospitalized at the time of the study. The diagnosis of celiac disease was made on the basis of an intestinal biopsy and a positive response to the lymphocyte inhibition factor (LIF) test, as well as a good response to withdrawal of gluten from the diet. HLA-B8 was found in 10 of the 18 celiac patients (56%). Of the 27 nonceliac children, three (11%) had HLA-B8. The LIF-positive celiac group had an HLA-B8 frequency five times greater than that in the nonceliac group, confirming our previous finding that the LIF test is a good diagnostic criterion for celiac disease. The combination of an intestinal biopsy, the LIF test and the presence of HLA-B8 could be a useful triad for diagnosis of celiac disease.

Celiac Disease↗
PubMed2003

Lack of endoscopic visualization of intestinal villi with the "immersion technique" in overt atrophic celiac disease.

BACKGROUND: The endoscopic appearance of the duodenal folds can predict the presence of celiac disease. However, endoscopic alterations can be minimal and the disease can have a "patchy" distribution histopathologically. The observation that intestinal villi can be better visualized when the duodenum is filled with water led to the development of an endoscopic "immersion technique" to assess celiac disease. METHODS: Endoscopy with duodenal biopsies was performed in 20 patients with malabsorption syndrome (positive for antiendomysial antibodies) and in 30 patients with reflux-like symptoms (negative for antiendomysial antibodies). Duodenal hypotonia was induced pharmacologically, water was introduced, and the mucosa was observed for the presence of villi. Photographs were obtained for subsequent analysis. The endoscopic appearance was classified from 1 (folds certainly present) to 4 ("scalloped valvulae"); villous structures were classified from 1 (definitely present) to 3 (definitely absent). RESULTS: Celiac disease was confirmed histopathologically in all patients with positive antiendomysial antibodies. The endoscopic appearance of the duodenum with air insufflation alone had a positive predictive value for the diagnosis of celiac disease of 84% and a specificity of 87%. Visualization of villi with the "immersion technique" had a higher positive predictive value (99%) and specificity (99%). CONCLUSIONS: A lack of visualization of intestinal villi in the descending duodenum with the "immersion technique" may increase the diagnostic accuracy of endoscopy for celiac disease. This technique could also be useful for targeting duodenal biopsies.

Adult↗