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Benign pneumatosis intestinalis in the setting of celiac disease.

Pneumatosis intestinalis is an uncommon finding that may indicate the presence of several alarming pathological conditions, including bowel ischemia, that require urgent surgical intervention. We report the case of a 51-year-old man with celiac disease who underwent resection of a large duodenal adenocarcinoma. Although he initially recovered rapidly from his procedure, he subsequently developed abdominal distention and leukocytosis. Abdominal imaging revealed extensive small bowel pneumatosis and pneumoperitoneum. Emergent surgical exploration revealed only bowel wall air cysts and dilated bowel but failed to demonstrate any intra-abdominal pathology. The patient recovered uneventfully and was discharged without any further complications or recurrence of symptoms. We review the current literature on the rare finding of pneumatosis intestinalis in the setting of celiac disease. In all reported cases, even when pneumatosis is accompanied by pneumoperitoneum, these alarming findings have proved to be of "benign" origin, that is with no evidence of bowel ischemia, perforation, or peritonitis. The available evidence suggests that pneumatosis in the setting of celiac disease may reflect the dissection of intraluminal gas into the inflamed bowel wall without accompanying intra-abdominal pathology. We conclude that pneumatosis intestinalis, even with accompanying pneumoperitoneum, does not uniformly mandate surgical exploration in patients with celiac disease.

Adenocarcinoma↗

Glomerular IgA deposits in patients with celiac disease.

Glomerular immunopathology was studied in 25 patients with newly diagnosed celiac disease. None had clinical signs of renal disease. Glomeruli were obtained by fine-needle aspiration biopsy. The specimens were processed and studied by indirect immunofluorescence for immunoglobulins and complement. Mesangial IgA was found in 8 of the patients. It occurred occasionally together with slight IgG or IgM, but C3 was not seen in these patients. IgA-class circulating immune complexes (CIC), antireticulin antibodies (ARA), antigliadin antibodies (AGA), and rheumatoid factor (RF) occurred significantly more often in the patients with mesangial IgA than in the 17 patients having no mesangial IgA. The patients with mesangial IgA also had significantly higher mean levels of serum IgA, IgA-ARA and IgA-AGA than those without. The results suggest that glomerular mesangial deposits of IgA occur frequently in untreated celiac disease and that they are in some way associated with circulating IgA-class antibodies and immune complexes. In this situation IgA seems to be deposited without being able to induce clinically overt glomerulonephritis, a circumstance that may be related to the lack of complement in the deposits.

Adolescent↗

Holmes-Adie syndrome, autoimmune hepatitis and celiac disease: a case report.

A 35-year-old female patient presented with the following symptoms of Holmes-Adie syndrome: photophobia,enlargement of the left pupil unresponsive to light, Achilles areflexia. The pilocarpine test was positive. No tumor or other neurological abnormality was found. She had a 19-year history of autoimmune hepatitis. Flares up were observed following each 3 deliveries. At age of 31 she presented with diarrhea and weight loss. Abdominal tumor was detected by ultrasound. The surgically removed tumor was histologically a benign mesenteric multicystic lymphangioma.Simultaneously, celiac disease was diagnosed.Gluten-free diet resulted in a significant improvement of celiac disease,but not of autoimmune hepatitis. Autonomic neuropathy was proven by standard cardiovascular tests. The patient was a homozygous carrier for HLA DQ2 antigen characteristic for celiac disease and heterozygous for HLA DR3 B8 frequent in autoimmune liver diseases. Our novel observation on association of Holmes-Adie syndrome with autoimmune hepatitis and celiac disease is suggestive for a common immunological background for all three entities present in a patient with mesenteric multicystic lymphangioma.

Adie Syndrome↗

Celiac disease in Brazilian adults.

UNLABELLED: Forty-eight adult patients with celiac disease between 15 and 68 years of age (mean, 41 years) were studied. Sixty-seven percent were female and 33% were male patients. Most of the patients were white (98%). The main clinical features were diarrhea (90%), weight loss (70%), and abdominal pain (56%). On physical examination, the main findings were pallor (40%), aphthous stomatitis (31%), and arthralgia (23%). Associated disorders included diabetes mellitus type I, osteoporosis, and atopy (6% each); dermatitis herpetiformis and depression (4% each); and hypothyroidism, hyperthyroidism, duodenal carcinoma, and Gilbert syndrome (2% each). The histologic results according to Marsh criteria (modified by Rostami) are as follows: type I, 10%; type II, 21%; type IIIa, 19%; type IIIb, 17%; and type IIIc, 33%. The sensitivity and specificity for the antiendomysium antibody-immunoglobulin A test were 92% and 100%, respectively, when considering the whole group of patients; however, the sensitivity (but not the specificity) decreased to 86% when taking into account only the group of patients with mild histologic alterations (Marsh type I, II, and IIIa). CONCLUSION: In general, the authors' results are similar to those described in developing countries, indicating that celiac disease might have the same spectrum of presentation regardless of the region studied.

Abdominal Pain↗

Characterization of cereal toxicity for celiac disease patients based on protein homology in grains.

BACKGROUND AND AIMS: Celiac disease is caused by T-cell responses to wheat gluten-derived peptides. The presence of such peptides in other widely consumed grains, however, has hardly been studied. METHODS: We have performed homology searches to identify regions with sequence similarity to T-cell stimulatory gluten peptides in the available gluten sequences: the hordeins of barley, secalins of rye, and avenins of oats. The identified peptides were tested for T-cell stimulatory properties. RESULTS: With 1 exception, no identical matches with T-cell stimulatory gluten peptides were found in the other grains. However, less stringent searches identified 11 homologous sequences in hordeins, secalins, and avenins located in regions similar to those in the original gluten proteins. Seven of these 11 peptides were recognized by gluten-specific T-cell lines and/or clones from patients with celiac disease. Comparison of T-cell stimulatory sequences with homologous but non-T-cell stimulatory sequences indicated key amino acids that on substitution either completely or partially abrogated the T-cell stimulatory activity of the gluten peptides. Finally, we show that single nucleotide substitutions in gluten genes will suffice to induce these effects. CONCLUSIONS: These results show that the disease-inducing properties of barley and rye can in part be explained by T-cell cross-reactivity against gluten-, secalin-, and hordein-derived peptides. Moreover, the results provide a first step toward a rational strategy for gluten detoxification via targeted mutagenesis at the genetic level.

Alkaloids↗

[Serological diagnosis of celiac disease: anti-gliadin peptide antibodies and tissue anti-transglutaminase].

Serological markers currently used for the diagnosis of celiac disease are anti-gliadin (AG) and anti-endomysium (AE) antibodies. Recently tissue transglutaminase (tTG) was identified as the specific autoantigen for endomysial antibodies. The aim of this work was to determine sensitivity and specificity of ELISA tests developed by using defined molecular structures as capture antigen for AG and AE antibodies. Three synthetic peptides, from the amino terminal region of alpha gliadin, were used as immobilized antigens for AG, and the transglutaminase from guinea pig liver for AE. A total of 80 sera from celiac patients, non celiac disease controls and healthy controls were examined. Age range was 7 months to 14 years. A sensitivity of 97% and a specificity of 86% was obtained for IgG determined by using as antigen one of the three synthetic peptides (corresponding to residues 31-55 of alpha gliadin). Therefore, this peptide appears as a highly sensitive antigen and more specific than gliadin. The best result, showing 100% of sensitivity and specificity, was obtained for IgA anti-tTG, thus pointing out the relevance of these antibodies as serological markers for celiac disease.

Adolescent↗

Adult celiac disease: what is the role of MRI?

PURPOSE: To evaluate the ability of MRI to identify intra- and extraintestinal findings of celiac disease in an adult population. MATERIALS AND METHODS: Forty-one subjects (18 men and 23 women; mean age = 41.3 years; 31 with biopsy-proven celiac disease, and 10 healthy volunteers) underwent MRI of the small bowel. MR studies were performed on a 1.5-T magnet using T2-weighted half-Fourier single-shot turbo spin-echo (HASTE) and true fast imaging in steady-state precession (True-FISP) sequences. The MR features and sensitivity, and the specificity and accuracy of some of these features are described. RESULTS: In the 31 celiac patients, MRI showed bowel dilatation in 61.3% (N = 19), increased number of ileal folds in 48.4% (N = 15), reversed fold pattern abnormality in 38.7% (N = 12), increased wall thickness in 16.1% (N = 5), duodenal stenosis in 6.5% (N = 2), intussusception in 12.9% (N = 4), mesenteric lymphadenopathy in 41.9% (N = 13), mesenteric vascular changes in 22.6% (N = 7), ascites in 6.5% (N = 2), and no abnormalities in 12.9% (N = 4). The volunteers had unremarkable exams. The overall specificity and accuracy were 100%, and sensitivity was 79% and 75% for increased number of ileal folders and reversed fold pattern abnormality, respectively. CONCLUSION: MRI is able to demonstrate intra- and extraintestinal features that may lead to the diagnosis of celiac disease in adults.

Adult↗

IgA anti-endomysial antibodies on human umbilical cord tissue for celiac disease screening. Save both money and monkeys.

Since celiac disease screening by traditional IgA anti-endomysial antibody test is limited by high costs of monkey esophagus commercial kits as well as by rising ethical problems related to the endangered species, the identification of an inexpensive and commonly available substrate for this antibody determination is urgently required. To achieve this goal, we compared the prevalence of IgA anti-endomysial antibodies detected on monkey esophagus with that on human umbilical cord. Fifty-seven (95%) of 60 untreated adult celiacs were positive for these antibodies on monkey esophagus as well as on human umbilical cord. IgA anti-endomysial antibodies, detected on both tissues, were negative in all 200 disease and healthy controls tested, displaying a 100% specificity for gluten-sensitive enteropathy. These data suggest that human umbilical cord can replace monkey esophagus for IgA anti-endomysial antibodies test. Human umbilical cord allows unlimited testing for celiac disease screening on wide series of high-risk subjects, permitting identification of greater numbers of asymptomatic celiac patients with a remarkable saving of money and bypassing the ethical problems related to killing monkeys.

Adult↗

Intestinal antibody pattern of celiac disease: occurrence in patients with normal jejunal biopsy histology.

BACKGROUND: Patients with celiac disease have immunoglobulin (Ig) M antibodies and IgA antigliadin antibody in gut secretions; this pattern of intestinal immunity may be a marker of latent celiac disease. Its frequency in patients referred for jejunal biopsy has been examined. METHODS: Serum IgG and IgA antigliadin antibody, jejunal fluid IgA and IgM antibodies to gliadin, ovalbumin and beta lactoglobulin, and jejunal fluid IgA and IgM concentrations were measured by enzyme-linked immunosorbent assay. RESULTS: Seventeen of 19 celiac patients on normal diet and 16 of 23 on gluten-free diet had the celiaclike antibody pattern, as did 41 of 217 other patients. Jejunal biopsy histology had been classified as normal in 38 of these, with minor abnormalities in 3; however, intraepithelial lymphocyte (IEL) counts were high in 13 cases. Trial of a gluten-free diet produced clinical improvement in 6 of 7 antibody-positive patients. After extra dietary gluten, one developed subtotal villous atrophy. CONCLUSIONS: The celiaclike intestinal antibody pattern and a high IEL count may be markers of latent gluten-sensitive enteropathy; some of these patients are clinically gluten sensitive in the absence of enteropathy.

Adolescent↗

Portal hypertension and nodular regenerative hyperplasia in a patient with celiac disease.

Nodular regenerative hyperplasia (NRH) is an uncommon cause of portal hypertension. The disease is often associated with rheumatological or lymphoproliferative disorders. We report the case of a 19-year old patient with celiac disease and portal hypertension. Biopsy of the liver revealed NRH as the underlying cause. There were no signs of autoimmune diseases or defects in the coagulation system that might cause NRH. Celiac disease is often associated with liver abnormalities, but the association with NRH has rarely been described. We discuss the possible relationship of celiac disease and NRH.

Adult↗

Celiac disease presenting as the Paterson-Brown Kelly (Plummer-Vinson) syndrome.

We describe two patients with Paterson-Brown Kelly (Plummer-Vinson) syndrome whose iron deficiency anemia was due to celiac disease. They presented with dysphagia 13 and 9 yr, respectively, before celiac disease was diagnosed. Neither had gastrointestinal symptoms suggestive of malabsorption. Celiac disease is a recognized cause of chronic iron deficiency and should be considered as an etiological factor for sideropenic dysphagia.

Celiac Disease↗

Celiac disease in an elite female collegiate volleyball athlete: a case report.

OBJECTIVE: To present the case of an elite female volleyball player who complained of diarrhea and fatigue after preseason training. BACKGROUND: The athlete lost 8.1 kg during the first 20 days of training, and we initially suspected an eating disorder. The sports medicine team interviewed the athlete and found she did not have psychological symptoms indicative of an eating disorder. The results of routine blood tests revealed critically high platelet counts; in conjunction with the physical findings, the athlete was referred to a gastroenterologist. DIFFERENTIAL DIAGNOSIS: Our initial suggestion was an eating disorder. Therefore, the differential diagnosis included anorexia athletica, anorexia nervosa, and bulimia nervosa. On referral, the differential diagnosis was anemia, gastrointestinal dysfunction, lymphoma, or bowel adenocarcinoma. Diarrhea, weight loss, and blood test results were suggestive of active celiac disease, and a duodenal biopsy specimen confirmed this diagnosis. TREATMENT: The athlete was treated with a gluten-free diet, which excludes wheat, barley, and rye. Dietary substitutions were incorporated to maintain adequate caloric intake. UNIQUENESS: The presence of active celiac disease may not be uncommon. However, elite athletes who face celiac disease present a new challenge for the athletic trainer. The athletic trainer can help guide the athlete in coping with the lifestyle changes associated with a gluten-free diet. CONCLUSIONS: One in every 200 to 400 individuals has celiac disease; many of these individuals are asymptomatic and, therefore, their conditions are undiagnosed. Undiagnosed, untreated celiac disease and patients who fail to follow the gluten-free diet increase the risk of further problems.

Journal Article↗

[Complication associated with celiac disease].

Enteropathy associated T cell lymphoma is the most serious complication of celiac sprue. The incidence of malignancy, in general, is approximately twofold greater in celiac disease than in the general population, but the risk of specific gastrointestinal malignancies is markedly increased. Lymphoma of the small intestine, comprises approximately 50% of the malignancies, and the most are of T-cell origin. We report a case of celiac disease associated T-cell Lymphoma of the jejunum in a woman. At the age of 33 the diagnosis of Celiac sprue was made, after institution of a gluten free diet the patient improved, but, fifteen year later, the patient begin with fever and abdominal pain. Laparotomy showed a perforation of the intestine by a tumour.

Celiac Disease↗

Atypical carcinoid tumor of the small bowel complicating celiac disease.

An atypical carcinoid tumor of the small bowel in a patient with celiac disease is described. Although an increased incidence of malignancy has been observed in celiac disease, this seems to be the first report of a carcinoid tumor of the small intestine associated with this condition. Although the significance of this association is unclear, the implications with regard to gastrointestinal endocrine cell proliferation and tumor histogenesis are discussed.

Carcinoid Tumor↗

The prevalence of celiac disease autoantibodies in patients with systemic lupus erythematosus.

OBJECTIVE: [corrected] Systemic lupus erythematosus has been associated with false positive autoantibodies for primary biliary cirrhosis, chronic active hepatitis, Sjogren's syndrome, rheumatoid arthritis, thyroid disorders, syphilis, and scleroderma. An increased prevalence of autoantibodies are found in celiac disease and systemic lupus erythematosus, which share the human lymphocyte HLA-B8 and HLA-DR3 histocompatibility antigens. This study examines the prevalence of celiac disease autoantibodies in systemic lupus erythematosus patients. METHODS: Patients observed in the Department of Rheumatology at our institutions in San Antonio, Texas with known systemic lupus erythematosus were offered participation in the study. One hundred three of the 130 patients contacted agreed to participate. Patients were excluded if they were pregnant or medically unable to undergo endoscopy. All volunteers were tested for the serological presence of IgA and IgM antigliadin and IgA antiendomysial antibodies. Those with positive serology underwent esophagogastroduodenoscopy with duodenal mucosal biopsy. RESULTS: Twenty-four of 103 (23.3%) systemic lupus erythematosus patients tested positive for either antigliadin antibody, whereas none of the 103 patients tested positive for antiendomysial antibody. None of the 24 antigliadin positive patients were found to have endoscopic or histological evidence of celiac disease, making the false positive rate of antigliadin antibody 23%. CONCLUSION: The presence of false positive antigliadin antibodies in patients with systemic lupus erythematosus is common. Despite shared human lymphocyte antigen loci there does not seem to be an association between celiac disease and systemic lupus erythematosus.

Autoantibodies↗

Paradoxical coexpression of proinflammatory and down-regulatory cytokines in intestinal T cells in childhood celiac disease.

BACKGROUND & AIMS: Specific T-lymphocyte reactions are central in the pathogenesis of celiac disease, an inflammatory small-bowel enteropathy caused by a permanent intolerance to gluten. To delineate local T-lymphocyte responses to gluten, the cytokine expression in jejunal T lymphocytes of pediatric celiac patients with active disease, i.e., untreated and gluten-challenged celiac patients, was determined and compared with that of treated, symptom-free celiac patients and controls. METHODS: Biopsy samples were collected from celiac patients and controls. Intraepithelial and lamina propria T lymphocytes were isolated separately, and the cytokine messenger RNA levels were determined by using quantitative real-time reverse-transcription polymerase chain reaction. Interferon (IFN)-gamma and interleukin (IL)-10 were determined at the protein level by immunohistochemistry. RESULTS: Active celiac disease was characterized by distortions in cytokine expression by T lymphocytes, with highly significant increases of IFN-gamma and IL-10 but no concomitant increases in tumor necrosis factor alpha, transforming growth factor beta1, or IL-2 and no induction of IL-4. A marked shift of IFN-gamma and IL-10 production from the lamina propria to the epithelium was characteristic of active celiac disease, and as many as one fourth of the intraepithelial lymphocytes expressed IFN-gamma. Intraepithelial T lymphocytes in treated, symptom-free celiac patients still had increased IFN-gamma levels compared with controls. CONCLUSIONS: In celiac patients, gluten intake seems to cause an overreaction in intraepithelial T lymphocytes, with uncontrolled production of IFN-gamma and IL-10. This may cause both recruitment of intraepithelial lymphocytes and a leaky epithelium, leading to a vicious circle with amplified immune activity and establishment of intestinal lesions.

Adolescent↗

Cerebellar syndrome in adult celiac disease with vitamin E deficiency.

We studied a woman with adult onset celiac disease complicated by a cerebellar syndrome that progressed despite the resolution of the malabsorption symptoms with a gluten free diet. The patient presented vitamin E deficiency and the cerebellar symptoms improved with vitamin E therapy. This case supports the possible role of this deficiency in the development of the neurological complications of celiac disease.

Adult↗

Small-bowel mucosa in asymptomatic children with celiac disease. Mucosal changes with gluten-free diets.

Even though children with celiac disease may appear to be progressing satisfactorily while taking gluten-free diets, dietary lapses and persisting mucosal abnormalities are common. Of 32 children with celiac disease proven by biopsy only ten kept regularly to their diet, and 11 continued to ingest gluten regularly. Despite satisfactory clinical progress, intestinal biopsy specimens obtained after at least one year of a gluten-free diet were markedly abnormal in eight and were normal in only 14 children. Laboratory criteria also failed to detect those with persisting mucosal abnormalities. The only way to ensure that the small-bowel mucosa returns to normal and remains so is by intestinal biopsy.

Adolescent↗