IgA Tissue Transglutaminase Antibodies Research Articles

Celiac disease prevalence in patients with iron deficiency anemia of obscure origin

Anemia, especially due to iron deficiency, is a frequent feature in celiac disease. In this study, we aimed to define the prevalence of celiac disease in Turkish patients with iron deficiency anemia of obscure origin. One thousand four hundred and eighty-six consecutive patients with iron deficiency anemia were evaluated for etiology. Of those, 77 patients were found to have iron deficiency anemia of obscure origin. Sera from 77 patients with iron deficiency anemia of obscure origin and 119 healthy controls were tested for IgA and IgG tissue transglutaminase (tTG) antibodies by ELISA. Endoscopic mucosal biopsies were taken from the second part of the duodenum in these patients. Histopathologic examination results of patients were stratified according to Marsh classification. IgA and IgG class anti-tTG antibodies were found positive in 6 (7.8%) and 3 (3.9%) patients with iron deficiency anemia of obscure origin, respectively. Three patients had only IgA anti-tTG and 3 had both IgA and IgG anti-tTG antibodies. In the control group, 1 subject was positive for both IgA and IgG anti-tTG antibodies (0.7%). Six patients (7.8%) and 1 control subject (0.8%) had histopathologic findings of celiac disease (p=0.02). Patients with iron deficiency anemia of obscure origin had increased prevalence of celiac disease. Our study results suggest that serological screening may be recommended for early detection of celiac disease in patients with iron deficiency anemia of obscure origin.

Neue Aspekte der Antikörperbestimmung zur Zöliakiediagnostik

Antibody assays play an important role in the diagnosis of coeliac disease (coeliac sprue, gluten-sensitive enteropathy), a condition characterized by immunological intolerance to gluten from wheat and from proteins of related cereals in genetically predisposed persons. Enhanced concentrations of IgA-antibodies against tissue transglutaminase or endomysium under gluten-containing normal diet represent an important indication for a biopsy from the small intestine. Demonstration of typical changes in the mucose of the small intestine is still required for the definitive diagnosis of coeliac disease. Recently highly specific antibodies against deamidated gliadin peptides were detected in serum. These antibodies further improve the reliability of serologic diagnosis. The new assays for IgG-antibodies against deamidated gliadin peptides have a very high diagnostic accuracy and are comparable to IgA tissue transglutaminase antibodies. Further investigations have to show whether IgG-antibodies against deamidated gliadin peptides are a reliable disease marker also in case of IgA deficiency. Prospective studies are needed to show whether antibody assays could replace biopsy in the diagnosis of coeliac disease in a substantial number of patients.

Adult celiac disease with acetylcholine receptor antibody positive myasthenia gravis

Celiac disease has been associated with some autoimmune disorders. A 40-year-old competitive strongman with celiac disease responded to a gluten-free diet, but developed profound and generalized motor weakness with acetylcholine receptor antibody positive myasthenia gravis, a disorder reported to occur in about 1 in 5000. This possible relationship between myasthenia gravis and celiac disease was further explored in serological studies. Frozen stored serum samples from 23 acetylcholine receptor antibody positive myasthenia gravis patients with no intestinal symptoms were used to screen for celiac disease. Both endomysial and tissue transglutaminase antibodies were examined. One of 23 (or, about 4.3%) was positive for both IgA-endomysial and IgA tissue transglutaminase antibodies. Endoscopic studies subsequently showed duodenal mucosal scalloping and biopsies confirmed the histopathological changes of celiac disease. Celiac disease and myasthenia gravis may occur together more often than is currently appreciated. The presence of motor weakness in celiac disease may be a clue to occult myasthenia gravis, even in the absence of intestinal symptoms.

Restless leg syndrome in a patient with celiac disease: a coincidence or an association?

To the Editor: A myriad of extraintestinal manifestations of celiac disease have been described,1 but none have included restless leg syndrome (RLS). We report a case of celiac disease presenting with RLS. A 45-year-old woman was seen in June 2007 for exacerbation of sleep discomfort related to an uncomfortable sensation in the limbs at night that also resulted in daytime somnolence. The sensation decreased with movement and disappeared with walking. She also reported abdominal pain and diarrhea. Repeated colonoscopies in 2002 and 2006 were normal. Her medical history revealed a long-standing anemia refractory to oral iron supplementation and weight loss. A neurological exam was normal. Initial laboratory studies showed a hemoglobin of 9.8 g/dL (reference range, 13-15 g/dL) with a mean corpuscular volume of 82 fL, a mean corpuscular hemoglobin of 26.5 pg/cell (reference range, 3033 pg/cell) serum iron of 11.3 μmol/L (normal range, 8.8-32.4 μmol/L) and serum ferritin of 6.2 ng/mL (normal range, 30-400 ng/mL). Thyroid stimulating hormone, folic acid and vitamin B12 levels were normal. Her 25-hydroxyvitamin D was 7.2 ng/mL (normal, >20 ng/mL). Bone mass densitometry revealed osteoporosis at the spinal level (T score, −3.2). IgA endomysial antibodies and IgA tissue transglutaminase antibodies (>200 RU/mL, normal <20 RU/mL), and IgA antigliadin antibodies (30 UI/mL, normal <5 UI/mL) were positive. Upper gastrointestinal endoscopy revealed an atrophic duodenal mucosa. Duodenal biopsy showed severe villous atrophy with mononuclear infiltrate and epithelial cell damage. A diagnosis of celiac disease with secondary RLS was made. A gluten-free diet and intravenous iron supplementation abated the symptoms of RLS in 6 weeks. At the 6-month visit she had gained 8 kilograms and her serum ferritin level was 57 ng/mL. The patient was diagnosed as having secondary RLS and received parenteral iron supplementation for 5 days.

Pre-endoscopic screening for Helicobacter pylori and celiac disease in young anemic women

To evaluate the usefulness of pre-endoscopic serological screening for Helicobacter pylori (H pylori) infection and celiac disease in women aged < 50 years affected by iron-deficiency anemia (IDA). One hundred and fifteen women aged < 50 years with IDA were tested by human recombinant tissue transglutaminase IgA antibodies (tTG) and anti-H pylori IgG antibodies. tTG and H pylori IgG antibody were assessed using an enzyme-linked immunosorbent assay (ELISA). All women were invited to undergo upper GI endoscopy. During gastroscopy, biopsies were collected from antrum (n = 3), gastric body (n = 3) and duodenum (n = 4) in all patients, irrespective of test results. The assessment of gastritis was performed according to the Sydney system and celiac disease was classified by Marsh's System. 45.2% women were test-positive: 41 patients positive for H pylori antibodies, 9 patients for tTG and 2 patients for both. The gastroscopy compliance rate of test-positive women was significantly increased with respect to those test-negative (65.4% vs 42.8%; Fisher test P = 0.0239). The serological results were confirmed by gastroscopy in 100% of those with positive H pylori antibodies, in 50% of those with positive tTG and in 81.5% of test-negative patient. Sensitivity and specificity were 84.8% and 100%, respectively for H pylori infection and, 80% and 92.8% for tTG. Twenty-eight patients had positive H pylori antibodies and in all the patients, an active H pylori infection was found. In particular, in 23 out of 28 (82%) patients with positive H pylori antibodies, a likely cause of IDA was found because of the active inflammation involving the gastric body. Anti-H pylori IgG antibody and tTG IgA antibody testing is able to select women with IDA to submit for gastroscopy to identify H pylori pangastritis and/or celiac disease, likely causes of IDA.

A novel one-hour13C-sorbitol breath test versus the H2-sorbitol breath test for assessment of coeliac disease

Objective. The H2-sorbitol breath test (H2-SBT) has previously been suggested as a screening tool for coeliac disease. We developed an alternative 13C-sorbitol breath test (13C-SBT). The aim of the study was to compare the diagnostic properties of the H2-SBT and the 13C-SBT in a clinical setting. Material and methods. Thirty-nine coeliac patients, 40 patient controls (mainly patients with irritable bowel syndrome) and 26 healthy volunteers underwent the breath tests. The patients were given an oral load of 5 g sorbitol and 100 mg 13C-sorbitol dissolved in 250 ml tap-water. H2, CH4 and 13CO2 concentrations were measured in end-expiratory breath samples every 30 min for 4 h. Increased H2 concentration ≥20 ppm from basal values was used as the cut-off for the H2-SBT. Results. The H2-SBT had a sensitivity of 71%, a specificity of 46% versus healthy controls, and a specificity of 25% versus patient controls. Individuals with methane-producing intestinal flora had significantly lower peak H2 concentrations than non-methane producers. The 13C-SBT reached maximal combined sensitivity/specificity (74%/85%) for both control groups after 1 h. A diagnostic algorithm which stratified patients into high-, moderate- and low risk for coeliac disease was proposed. Following the algorithm, 62% of coeliac patients were detected with 100% specificity. The 13C-SBT, but not the H2-SBT, correlated with age and serum IgA tissue-transglutaminase antibody levels in coeliac patients. Conclusions. The novel 13C-SBT has superior diagnostic properties compared to the H2-SBT, which has unsatisfactory specificity in clinical practice. The 1-h 13C-SBT may be a useful supplemental test when investigating for coeliac disease.

The Relationship between Autoimmune Bullous Disease and Systemic Disorders

The mystery of the association of skin disease with systemic disease has been a matter of fascination to dermatologists for hundreds of years. There are a myriad of articles and case reports linking various cutaneous findings to internal disease. The implication has always been that some soluble or cellular factor is being produced that results in a cutaneous disease. Immunobullous disease associates a particular antibody with a cutaneous disease and so the responsible factor for associating an immunobullous disease with a systemic disease is production of an antibody. We will review the milestones in immunobullous disease that have refined our understanding of these associations and consider possible pathogenic mechanisms. Dermatitis herpetiformis (DH) is a papulovesicular skin disease with gluten-sensitive enteropathy (celiac disease, CD) and a unique HLA type (HLADQ2 and DQ8) associated in virtually all cases. Granular deposition of IgA is the immunopathological hallmark. Marks et al. (1966) are generally credited with describing the association of CD with DH. Marks, Fry, and others established that the skin disease with its IgA deposition as well as the intestinal disorder responded to gluten restriction and recurred with the reinstitution of a gluten-containing diet culminating in a landmark paper on gluten challenge in 1983 (Leonard et al., 1983). Katz and Strober (1978) clarified the association of HLA B8/DR3 with the entire spectrum of CD with DH. Kumar et al. (1987) established the presence of IgA endomysial antibodies in DH and CD patients. The endomysial antigen was found to be tissue transglutaminase, and now testing for IgA tissue transglutaminase antibody has become the standard for the serological diagnosis of CD. The link between the intestine and the skin in genetically predisposed individuals is twofold. First, Hall et al. (2007) have established that cytokines elaborated at the site of intestinal inflammation are likely critical to the inflammatory process in the skin. Second, Sardy et al. (2002) have shown that epidermal transglutaminase is the antigen bound to the IgA stimulated by the intestinal inflammatory process. The association between DH and thyroid disease is even more intricate. In 1983, Cunningham and Zone (Cunningham and Zone, 1985) closely evaluated 50 DH patients, and showed that these patients had a high incidence of thyroid autoantibodies as well as hyperthyroidism, hypothyroidism, and thyroid enlargement. Gaspari et al., 1990. showed that this association was not related to the underlying HLA B8/DR3 genotype but was likely related to a high prevalence of Hashimoto’s thyroiditis manifested by thyroid autoantibodies and was independently associated with DH. Whether or not the intestinal inflammation in DH patients is responsible for stimulating the thyroid autoimmune process by a cellular or humoral mechanism remains to be determined (Gaspari et al., 1990). Since the 1980s, several immunobullous disorders have been linked to inflammatory bowel disease including bullous pemphigoid, epidermolysis bullosa acquisita (EBA), and linear IgA bullous disease in case reports and case series. Of these, EBA appears to have the strongest association with inflammatory bowel disease. EBA, an immunobullous disorder is characterized by autoantibodies to type VII collagen, the major structural protein of anchoring fibrils, and clinical presentations resembling bullous pemphigoid, cicatricial pemphigoid, or epidermolysis bullosa. EBA has been described in patients with both Crohn’s disease and UC, with Crohn’s disease being the predominant association. In a study of 51 EBA patients, Chen et al. (2002) found that 25% (13 of 51) had inflammatory bowel disease: 12 had Crohn’s disease and 1 had UC. They also demonstrated full-length (290 kDa) type VII collagen in normal colonic extracts from four patients and type VII collagen immunoreactivity in the colonic basement membrane. Of 19 patients, 13 patients with Crohn’s disease and 4 of 31 patients with UC were found to have elevated levels of circulating antibodies to the immunodominant NC1 domain of type VII collagen by ELISA. Despite these findings, there is no evidence linking an autoantibody to the pathogenesis of inflammatory bowel disease. The presence of type VII collagen antibodies is thought to represent epitope spreading, resulting from exposure of type VII collagen epitopes due to damage to the overlying colonic mucosa. Immunobullous diseases have also been linked to malignancies. The quintessential disorder is paraneoplastic pemphigus (PNP), described by Anhalt et al. (1990). PNP is characterized by painful mucosal erosions and polymorphous

Health Care Providersʼ Knowledge About Celiac Disease

Purpose: Celiac disease (CD) is estimated to occur in ∼1% of the United States population, however, the disease remains mostly undiagnosed. The reasons for this are unclear, though it may be due to lack of knowledge about CD among medical practitioners. Methods: We administered an eleven question survey to internal medicine residents and attendings and gastroenterology fellows and attendings at a large university medical center, and to family practice physicians in New York State. The questionnaire included questions about the prevalence, associated conditions and presenting symptoms, diagnosis, and management of CD. Results: 325 questionnaires were completed. The average scores for the different groups were: interns 40.0%, junior residents 46.5%, senior residents 52.9%, internal medicine attendings 54.6%, gastroenterology fellows 65.4%, gastroenterology attendings 66.2%, and family practice physicians 46.4%. Analysis of variance demonstrated that the overall test scores differed significantly among the seven groups (P < .001). GI attendings and GI fellows scores were statistically equivalent, but differed significantly from those of IM residents and attendings and family practice attendings, therefore gastroenterologists were compared to IM/family practice physicians for comparisons based on level of training. Only 19% of respondents knew the prevalence of CD in the U.S. There was a significant association between level of training and knowledge of the prevalence of CD (P= 0.002). 35% of respondents answered that IgA tissue transglutaminase or IgA endomysial antibodies were the most sensitive and specific diagnostic tests. Again, there was a significant association based on level of training (P= < 0.0001). Gastroenterologists also scored better on the questions that asked about associated diseases, lab abnormalities, and extraintestinal manifestations of CD. However, there was no difference in knowledge of GI symptomatology of CD between the groups. See Table 1 for full results.Table 1: Individual Group's Score for Each TopicConclusion: The overall knowledge about CD is poor, even among gastroenterologists, though is more pronounced in non-gastroenterologists. Level of knowledge of diagnostic testing and prevalence are associated with level of training, while knowledge of clinical presentations is not. Unless there are more efforts to increase knowledge of CD among all medical practitioners, especially primary care providers, the disease will continue to be underrecognized.

Increased Risk for Coeliac Disease in Paediatric Patients with Migraine

The aim was to determine the prevalence of coeliac disease (CD) in paediatric patients with migraine. Serum tissue transglutaminase IgA (tTGA) antibodies and IgA concentrations were measured in 73 patients with migraine (age range 6-17 years) and the control group (n = 147). Patients having positive tTGA antibodies underwent duodenal biopsy. Four patients (5.5%) from the study group and one (0.6%) from the control group had positive tTGA antibody titres (P < 0.05). Three patients with migraine had normal duodenal histology and were considered as potential CD. One patient from the study group and one from the control group declined to have biopsy. tTGA antibody is considered as a reliable indicator for the presence of CD. However, some patients with positive antibodies may have normal biopsy initially and are classified as having potential CD. Our finding of a higher prevalance of tTGA antibodies in paediatric migraine patients suggests that an association between migraine and CD might exist.

The Prevalence of Celiac Disease in Blood Donors in Iceland

Prospective epidemiological studies based on serological methods have shown that celiac disease is more common than previously thought. The aim of this study was to evaluate the prevalence of celiac disease among apparently healthy blood donors in Iceland. Plasma samples were obtained from 813 apparently healthy blood donors at the FSA Hospital Blood Bank in Akureyri, Iceland, between December 2004 and January 2007 and screened for human tissue transglutaminase IgA antibodies. Positive samples were retested and, if the test was again positive, the subject was referred to a gastroenterologist for clinical examination and a duodenoscopy with mucosal biopsies. Six subjects tested positive for tissue transglutaminase. The prevalence of biopsy-confirmed celiac disease, according to modified Marsh classification, among apparently healthy blood donors in Iceland was found to be 1:136 (0.74%, 95% confidence interval 1/667-1/75, 0.15-1.33%). Prevalence of celiac disease in Iceland is similar to what has been reported in many other countries.

Strongly Positive Tissue Transglutaminase Antibodies are Associated With Marsh 3 Histopathology in Adult and Pediatric Celiac Disease

Our objective was to determine whether high serologic IgA tissue transglutaminase antibodies (TTGA) are exclusively associated with celiac disease (CD). IgA TTGA are found in the serum of most individuals with CD. This serologic marker is used to screen individuals with suspected CD for duodenal biopsy, the gold standard of CD diagnosis. Data suggest strongly positive IgA TTGA >or= 100 units are highly specific for CD histopathology in pediatric patients and may be sufficient for diagnosis. Records of adult and pediatric subjects in the celiac study at the University of Utah and University of California Irvine were reviewed for strongly positive TTGA. Pathology reports from duodenal biopsies of subjects with IgA TTGA >or= 100 units were graded as 0 to 3 by modified Marsh criteria. From a pool of 1882 subjects with IgA TTGA assayed, 208 had IgA TTGA >or= 100 units. Seventy-six of these, including 28 children and 48 adults, also had duodenal biopsies. Villous atrophy (Marsh 3 histopathology) was found on biopsy in 73 (96%) of these subjects. The remaining 3 subjects had intermediate Marsh histology. One (Marsh 1) had a complete serologic response to a gluten-free diet and 2 had Marsh 2 lesions and positive endomysium, making early CD most likely. IgA TTGA >or= 100 units occur almost exclusively in the setting of Marsh 3 duodenal histopathology in adults and children. Rare cases without villous atrophy were marked by intermediate Marsh changes suggestive of early CD. IgA TTGA >or= 100 arbitrary units indicate duodenal changes consistent with CD.

Tissue Transglutaminase Antibodies Are a Useful Serological Marker for the Diagnosis of Celiac Disease in Patients With Down Syndrome

Celiac disease (CD) is overrepresented among patients with Down syndrome (DS), who frequently lack any typical symptoms. Therefore, screening for CD is recommended in this high-risk group. The aim of the study was to determine the prevalence of CD in Arab children with DS and evaluate the contribution of immunoglobulin (Ig) A and IgG anti-gliadin antibodies (AGA), IgA and IgG tissue transglutaminase (TTG) antibodies, and IgA anti-endomysial antibodies (EMA) to screen for CD in children with DS. A total of 52 Arab patients with DS and 52 healthy Arab control subjects were studied for CD using various serological markers. Data on age, sex, weight, height, gastrointestinal symptoms, and endocrine abnormalities were recorded. Human leukocyte antigen (HLA) was studied in patients undergoing small intestinal biopsy. Five patients with DS were IgA TTG-positive and only 1 patient with DS was IgG TTG-positive. EMA was negative in all patients with DS. TTG (IgA and IgG) and EMA were negative in all control children. IgA AGA was positive in 12 patients with DS and 3 control subjects (P = 0.02), whereas IgG AGA was positive in 41 patients with DS and 26 control subjects (P = 0.004). Only children testing positive for TTG underwent upper endoscopy with duodenal biopsy. Two children with DS were diagnosed with CD. Both patients were IgA TTG-positive. One was HLA DQ2-positive and another was negative for HLA DQ2 and DQ8. CD is prevalent (3.8%) in Arab patients with DS. Based on our cohort, IgA TTG is useful in diagnosing patients with CD and DS.

Reduced Chemokine Receptor 9 on Intraepithelial Lymphocytes in Celiac Disease Suggests Persistent Epithelial Activation

Celiac disease is caused by an inappropriate immune response to dietary gluten, with increased epithelial lymphocyte infiltration in the duodenum/jejunum as a hallmark. The chemokine receptor 9 (CCR9) is a small intestinal homing receptor normally found on most mucosal T cells in this organ. Because CCR9 expression appears to be activation dependent, we examined CCR9 on duodenal T cells from untreated and treated (gluten-free diet) patients with celiac disease and healthy controls. Duodenal biopsy specimens and blood samples were obtained for histologic analysis and flow-cytometric CCR9 analysis of isolated lymphocytes. CCR9 expression after activation was studied in peripheral blood T cells from healthy volunteers. The median number of CCR9(+) cells among CD3(+) T cells in epithelium and lamina propria, respectively, was 56% and 48% in controls, 11% and 40% in treated patients, and 1% and 8% in untreated patients. Significant differences occurred between controls and treated or untreated patients in the epithelium but only between controls and untreated patients in the lamina propria (P=.008, all comparisons). No such differences were seen in peripheral blood, but stimulation with phorbol myristate acetate and ionomycin and, to a lesser extent, stimulation via NKG2D reduced the CCR9 expression on blood T cells. CCR9 expression is reduced on epithelial and lamina propria T cells in untreated celiac disease. Down-regulation of CCR9 persists in intraepithelial T cells from well-treated patients. This suggests ongoing immune activation preferentially within the epithelium.

Celiac disease: Association with adult-onset &lt;i&gt;Still's disease&lt;/i&gt;: Apropos of a clinical case

Adult-onset Still's disease (AOSD) is a rheumatic disorder of unknown etiology characterized by a triad of fever, polyarthritis and evanescent rash. We present a case report of a 28-year-old female who presented with complaints of fever, joint pains, rash, weakness for the past 4 years and diarrhea for the past 2 years. On investigation the patient was diagnosed to be a case of AOSD. Duodenal biopsy report was suggestive of celiac disease with a positive IgA tissue transglutaminase and anti-endomysial antibody. The patient was started on weekly methotrexate and gluten-free diet and her symptoms gradually improved. The patient remains in our follow-up and is doing well.

Prevalence of coeliac disease in patients with autoimmune thyroiditis in a Turkish population

To investigate the prevalence of coeliac disease in a series of Turkish patients with autoimmune thyroiditis. Sera from 136 consecutive patients with newly diagnosed autoimmune thyroiditis and 119 healthy blood donors were tested for IgA tissue transglutaminase antibody with enzyme-linked immunosorbent assay. Endoscopic mucosal biopsy from the second part of duodenum was performed in patients with positive antibody test. Eight patients (5.9%) and one control subject (0.8%) were positive for IgA tissue transglutaminase antibody (OR: 7.38, 95% CI: 0.91-59.85, P = 0.04). Six patients and one control agreed to take biopsies. Histopathological examination revealed changes classified as Marsh IIIa in one, Marsh II in one, Marsh I in two, and Marsh 0 in two patients with autoimmune throiditis, and Marsh I in one blood donor. Turkish patients with autoimmune thyroiditis have an increased risk of coeliac disease and serological screening may be useful for early detection of coeliac disease in these patients. Our findings need to be confirmed in a larger series of patients.

Autoimmune diseases in Turner syndrome

It has been known that females with Turner syndrome (TS) have an increased prevalence of autoantibodies and are at increased risk of developing autoimmune diseases. Immunological disturbances have been described in TS: a slight decrease in immunoglobulin serum levels and in circulating T and B cells percentages. This data is not entirely in concordance with some more recent studies. The effects of the immune derangement, found only by some studies, may account for the association of TS with autoimmune disease. In TS there is an increased risk of celiac disease (CD), though the risk is considerably smaller than that for thyroiditis. Some multicenter studies have been performed (Sweden, Canada, Poland, Italy and Germany) and the reported prevalence of CD is 4.2–6.4% in TS versus 0.35–0.5% in the general population (GP). Apparently the risk is very low before school age. TS subjects with CD do not show particular dysmorphic signs. Only half of the CD subjects had a typical clinical picture and this finding speaks in favour of screening rather than just investigating TS patients with symptoms. In 44% of patients with CD and TS various autoimmune disorders were found vs. the 4.5–14% of CD subjects of the GP. In the subjects whose CD diagnosis was made before 15 years of age the autoimmune pathologies were found in 32% and in 55% in the subjects diagnosed afterward. Conclusions – As a high risk population TS girls and women should be screened for CD – if positive have diagnosis confirmed – according to North American Society for Pediatric Gastroenterology, Hepatology and Nutrition (NASPGHAN) guidelines, which represent the most up-to-date guidelines. Measurement of tissue transglutaminase IgA antibodies should begin at age 6 and repeated every 2–5 years. In TS subjects, positive to antibody determination, intestinal endoscopic biopsy was recommended, because in a third of CD subjects vascular alterations in the intestinal mucosa were detected. The screening for CD could be proposed as soon as possible after the diagnosis of TS. CD screening should be performed before the beginning of GH-therapy: to avoid a bad response to treatment, to improve growth and optimize bone mineral density.

Celiac disease: India on the global map

Celiac disease: India on the global map

Dermatitis herpetiformis and partial IgA deficiency

Although the specific IgA autoantibody responsible for the pathogenesis of dermatitis herpetiformis (DH) is unknown, the presence of IgA is considered essential in the pathogenesis of DH. To date, no cases of IgA deficiency have been reported in DH. In contrast, IgA deficiency is found in 2% to 3% of patients with celiac disease, a rate 10 to 15 times higher than the normal population. We report 2 patients with DH who also have partial IgA deficiency. We evaluated the sera of these patients for the presence of IgA autoantibodies to endomysium, tissue transglutaminase, epidermal transglutaminase, and gliadin. Both patients were found to have IgA endomysial and tissue transglutaminase antibodies, and serologic markers for DH. Corresponding IgG autoantibodies were not useful serologic markers of DH in the setting of IgA deficiency, as they often are in celiac disease. We then screened 98 DH sera for total IgA levels and identified 1 additional case with IgA deficiency. In conclusion, DH may develop in patients with partial IgA deficiency, indicating that pathogenically directed IgA antibodies are likely sufficient for cutaneous IgA deposition in this disease.

Latent and Potential Celiac Disease in Epileptic Turkish Children

In this study, we measured tissue transglutaminase IgA antibody for the serologic screening of celiac disease in 70 children with epilepsy. A pathologic titer of tissue transglutaminase IgA antibody was found in eight patients (4.7%). Small intestinal biopsies were performed in all antibody-positive patients, and two of them had small bowel histology typical of celiac disease (1.17%). Six had normal histology and were considered to have potential celiac disease. The prevalence of celiac disease in the epileptic group was 1.17%. None of the serum samples of 103 healthy children comprising the control group were positive for tissue transglutaminase IgA antibody. Therefore, we suggest that epileptic children be screened for celiac disease. (J Child Neurol 2006;21:6-7).

Antibodies to recombinant human tissue-transglutaminase in coeliac disease: Diagnostic effectiveness and decline pattern after gluten-free diet

Background/aims To assess the sensitivity and specificity of IgA and IgG tissue-transglutaminase antibodies assay, the pattern of antibody decline after gluten withdrawal and their modifications with reference to dietary compliance. Subjects We studied sera from 143 untreated coeliac children and adolescents (8.8 ± 6.1 years), 212 sera from 97 of those patients after gluten withdrawal, and 64 control subjects with non-coeliac intestinal disorders (6.8 ± 4.8 years). Methods Samples were tested for IgA and IgG class tissue-transglutaminase antibodies by radiobinding assay, using human-derived tissue-transglutaminase, and for IgA anti-endomysium antibodies by indirect immunofluorescence on monkey oesophagus. Results Untreated coeliac patients had significantly higher titres of IgA and IgG tissue-transglutaminase antibodies than controls ( p < 0.00001); the diagnostic sensitivity was 95.8% and 99.3%, respectively, and the specificity was 95.3%. Three patients with selective IgA deficiency were positive for IgG tissue-transglutaminase antibodies. The concordance rate between IgA tissue-transglutaminase antibodies and anti-endomysium antibodies was 98.1%. Patients on gluten-free diet showed a significant decrease in IgA and IgG tissue-transglutaminase antibodies with respect to untreated patients ( p < 0.0001). Tissue-transglutaminase was more sensible than anti-endomysium antibodies to detect small amounts of gluten intake when the compliance was poor. Conclusions The recombinant human tissue-transglutaminase antibodies assay is a highly sensitive and specific test for diagnosis of coeliac disease, and it is useful in monitoring the compliance to gluten-free diet.