Nature Reviews Gastroenterology & Hepatology, Jan 3, 2022
Coeliac disease is a systemic disorder characterized by immune-mediated enteropathy, which is cau... more Coeliac disease is a systemic disorder characterized by immune-mediated enteropathy, which is caused by gluten ingestion in genetically susceptible individuals. The clinical presentation of coeliac disease is highly variable and ranges from malabsorption through solely extra-intestinal manifestations to asymptomatic. As a result, the majority of patients with coeliac disease remain undiagnosed, misdiagnosed or experience a substantial delay in diagnosis. Coeliac disease is diagnosed by a combination of serological findings of disease-related antibodies and histological evidence of villous abnormalities in duodenal biopsy samples. However, variability in histological grading and in the diagnostic performance of some commercially available serological tests remains unacceptably high and confirmatory assays are not readily available in many parts of the world. Currently, the only effective treatment for coeliac disease is a lifelong, strict, gluten-free diet. However, many barriers impede patients’ adherence to this diet, including lack of widespread availability, high cost, cross-contamination and its overall restrictive nature. Routine follow-up is necessary to ensure adherence to a gluten-free diet but considerable variation is evident in follow-up protocols and the optimal disease management strategy is not clear. However, these challenges in the diagnosis and management of coeliac disease suggest opportunities for future research. Despite advances in testing for coeliac disease over the past two decades, awareness of this disease remains suboptimal worldwide. Here, Makharia and colleagues summarize the current knowledge of epidemiology, risk factors, diagnosis and follow-up of coeliac disease and highlight opportunities for future research. Coeliac disease is a global disease with a worldwide prevalence of around 1%. Most patients with coeliac disease are undiagnosed, misdiagnosed and/or experience a substantial delay in diagnosis, which suggests an inadequate awareness of the spectrum of its clinical manifestations. Despite improvements in the diagnosis of coeliac disease, persistent challenges include high inter-assay and intra-assay variation in serological test performance and high inter-observer variability in the grading of villous abnormalities. The optimal follow-up strategy for coeliac disease is unclear and studies are needed to define the timing and role of serological testing, gluten immunogenic peptide measurement and repeat biopsy. Increased access to dietitians, improved tools to assess adherence to a gluten-free diet, increased availability of gluten-free foods and reduced gluten contamination of gluten-free food products are needed. As maintaining strict adherence to a gluten-free diet is restrictive and challenging, the development of adjunct or alternative non-dietary therapies for coeliac disease is crucial. Coeliac disease is a global disease with a worldwide prevalence of around 1%. Most patients with coeliac disease are undiagnosed, misdiagnosed and/or experience a substantial delay in diagnosis, which suggests an inadequate awareness of the spectrum of its clinical manifestations. Despite improvements in the diagnosis of coeliac disease, persistent challenges include high inter-assay and intra-assay variation in serological test performance and high inter-observer variability in the grading of villous abnormalities. The optimal follow-up strategy for coeliac disease is unclear and studies are needed to define the timing and role of serological testing, gluten immunogenic peptide measurement and repeat biopsy. Increased access to dietitians, improved tools to assess adherence to a gluten-free diet, increased availability of gluten-free foods and reduced gluten contamination of gluten-free food products are needed. As maintaining strict adherence to a gluten-free diet is restrictive and challenging, the development of adjunct or alternative non-dietary therapies for coeliac disease is crucial.
Serology has significantly revolutionized the knowledge of celiac disease (CD), leading to the id... more Serology has significantly revolutionized the knowledge of celiac disease (CD), leading to the identification of unsuspected patients in at-risk CD groups, thereby increasing the number of CD diagnoses compared to the pre-screening era. Several markers for CD with a progressive diagnostic accuracy have been identified over the years, but only three of them, i.e. anti-tissue transglutaminase (anti-tTG), anti-endomysial (EmA) and anti-deamidated gliadin antibodies (DGP) are currently assessed in the daily clinical practice. A thorough review of the literature identified 44 original studies published between 1998 to 2022 for a total of 5098 pediatric and adult CD patients (without selective IgA deficiency) and 11930 disease controls. The results highlighted that anti-tTG IgA exhibited a higher sensitivity for CD (93.4%) than EmA IgA (92.8%), DGP IgG (81.8%) and DGP IgA (83.8%). The specificity of EmA IgA (99%) resulted to be higher than those of anti-tTG IgA (95.8%), DGP IgG (96.4%) and DGP IgA (92.1%). In patients with selective IgA deficiency, a condition closely related to CD, serological screening should include one of the three antibodies of IgG class, since anti-tTG, DGP and EmA have a very similar diagnostic accuracy in this clinical setting. According to age, there are two main diagnostic strategies for CD detection. In children, the revised ESPGHAN 2020 guidelines established that CD could be diagnosed in both symptomatic and asymptomatic children by high anti-tTG IgA titers (>10 times the cut-off) and EmA positivity with no need to obtain duodenal biopsy and HLA typing. In adult patients, although high tTG IgA titers (confirmed by EmA IgA positivity) correlate with villous atrophy, an intestinal biopsy is still considered mandatory for confirming CD diagnosis. Currently, a case finding approach in at-risk groups is preferred to mass screening for CD detection.
In previous study in our laboratory, we had shown the effect of radiation on morphometry of small... more In previous study in our laboratory, we had shown the effect of radiation on morphometry of small bowel of rats after 7 days of a single external doses of 1,000 rads with liver protection. Our aim was to determine the effect of the same procedure of gamma radiation in rats on the morphometry and D-Xylose absorption 7, 14 and 21 days after injury and compared with a control group matched by weight. The D-Xylose absorption was measured by plasmatic D-Xylose, 60' after an intragastric doses of the pentose (0.5 grs/kg of body weight). Results: In the control group the mucosal thickness was 880 +/- 40 mu (X +/- SD), the vellous height was 640 +/- 37.6 mu and the xylosemia 15.91 +/- 11.39 mg%. At 7 days in the irradiated group (n: 11) the weight decreased 33.36 +/- 6.29% (Vs. control p less than .001), the mucosal thickness and the vellous height were 666 +/- 97 (p less than .001) and 466 +/- 66.9 mu (p less than .001) and the xylosemia 6.16 +/- 2.3 (p less than .05). The figures at 14 days (n: 16) showed improving and completed recovery at 21 days (n: 14). Irradiated rats were shown to be affected in weight, morphology and D-Xylose absorption, they began recovering after 7 days, and completed recovery at 21 days.
Background: Refractory sprue (RS) is a rare and severe celiac-like enteropathy not responding to ... more Background: Refractory sprue (RS) is a rare and severe celiac-like enteropathy not responding to a strict gluten-free diet. Although prognosis is generally poor, little is known about the long-term outcome of patients. Aim: to report baseline characteristics and long-term outcome of a series of patients diagnosed and treated in a single institution. Materials: We report a retrospective cohort of 25 consecutive patients (15 females; mean age 46 yr; range 28-71) diagnosed with RS based on the presence of a non-responsive celiac-like enteropathy. All patients were intensively treated with a gluten-free diet, steroids, nutritional support and immunosupression. Results: Clinical and biological characteristics of patients suggest that, at least, 24 patients had clear evidences of celiac disease. HLA DQ2/DQ8 genes were present in all the 24 patients typed and autoimmune enteropathy was excluded in all. According to the genotyping, 12 patients had a polyclonal lymphocyte population (RS type I) and 13 exhibited monoclonal TCR-gamma gene rearrangements (RS type II). Sixteen patients had evidence of ulcerative jejunitis (UJ) (7 in RS type I and 9 in type II). Overall median follow-up time after diagnosis of RS was 29 mo/patient (range 7 to 204) (45 mo for type I and 24 mo for type II). Overall mortality was 48% (12 patients), 6 in each type. Eight patients with UJ (50%), 3 with lymphoma (two T-cell and one B-cell type) and 4 (44%) without ulcers died during follow-up. The causes of death were sepsis in the context of a progressive deterioration but without overt malignancies (n=5), vascular causes (n=3) and severe malnutrition (n=1). Three- and 5-yr survival rate after diagnosis of RS for the overall population was 60% and 56%. There was no differences between type I (67%, 58%) and type II RS patients (54% for both periods). Patients with UJ had lower but non-significant 3- and 5-yr survival rates (56% and 50%, respectively) compared with patients without ulcers (78% and 66%). Survivors had a favorable outcome. While 11 patients persists asymptomatic, two other cases still have mild diarrhea and one low body weight. Conclusions: We confirm that RS is a severe celiac disease-related disorder with very high mortality. Diagnosis of overt lymphoma (12%) in our long-term follow-up was not as frequent as was reported by other groups. A proportion of patients persist in good health for a long time irrespective of the nature of the IEL infiltration or the presence of UJ.
The World Gastroenterology Organisation aims to guide health providers in the best management of ... more The World Gastroenterology Organisation aims to guide health providers in the best management of celiac disease by providing a concise document with recommendations based on the latest evidence and resulting from our global expert consensus process on best current practices. In this guideline, a review team consisting of experts from 4 continents provides an updating with concepts that have been developed since the publication of the earlier guideline (2013). The guideline explores the epidemiology of the disorder from a global perspective and updates the diagnostic work-up as well as therapeutic and follow-up aspects of celiac disease. As a novelty in celiac disease, the team also provides cascades that represent a hierarchical set of diagnostic and management options to deal with the disease, ranked by available resources. The aim in presenting cascades is to highlight appropriate, context-sensitive, and resource-sensitive management options for all geographic regions, regardless of whether they are considered as “developing,” “semideveloped,” or “developed.” World Gastroenterology Organisation cascades are context-sensitive, and the context is not necessarily defined solely by resource availability. As first-line tests for symptomatic and asymptomatic patients, the experts suggest antitissue transglutaminase (antitTG) immunoglobulin A (IgA)+total IgA. However, to confirm a positive initial test, or in case of borderline tissue transglutaminase (tTG) IgA titers, it is recommended that other tests should be added, such as IgA antiendomysium, which is strongly recommended in most other guidelines. Other immunoassays regarded as good tests include the antiendomysium antibody (EMA) and immunoglobulin G-deamidated gliadin peptide (IgG-DGP) tests. A combination of IgG-DGP and IgA-tTG is particularly useful for detecting patients with celiac disease who are IgA-deficient. IgG-DGP is reported to be able to detect a few more IgA-sufficient patients who are missed on IgA-transglutaminase (IgA-tTG) tests. For some authors, performing IgA-tTG+IgG-DGP—2 tests addressing different antigens—is better than if 2 tests addressing the same autoantigen (IgA-tTG+IgA-EMA) are carried out. There was intense debate in the review team regarding the most appropriate serological strategy for diagnosing celiac disease, and we present readers with all of the various strategies here.
The present study was designed to determine the diagnostic usefulness of videoduodenoscopic inspe... more The present study was designed to determine the diagnostic usefulness of videoduodenoscopic inspection alone and the addition of vital dye staining in the detection of celiac disease. We additionally sought to evaluate interobserver agreement for specific duodenoscopic markers of mucosal atrophy. One hundred sixty-seven consecutive subjects who underwent duodenoscopy for intestinal biopsy were included in a prospective controlled study. Endoscopic examination was performed by experienced endoscopists according to a set protocol using methylene blue (1%) dye. All procedures were recorded on videotape, but only 20 (10 with atrophy and 10 normal) were used in a blinded, independent, randomized analysis by five reviewers to evaluate interobserver agreement. Endoscopic signs indicative of mucosal atrophy were as follows: reduction in the number or loss of Kerkring's folds, "scalloped" folds, "mosaic pattern," and visualization of the underlying blood vessels. Eighty-seven patients had celiac disease (57 newly diagnosed, 30 when treated). Seven treated patients had nonatrophic mucosa. In 80 patients the final diagnosis excluded celiac disease. Videoendoscopic inspection alone correctly identified 75 of 80 patients with complete mucosal atrophy and 86 of 87 with normal mucosa. False-negative diagnoses occurred in treated celiac patients with mild atrophy. Mosaic pattern (89%) and scalloped folds (86%) were the most useful endoscopic signs. Vital dye staining, as assessed by experienced endoscopists, provided identical results to those obtained by inspection alone. Sensitivity, specificity, and positive and negative predictive values for the presence of one or more than one feature were 94%, 100%, 100%, and 96%, respectively. The agreement (kappa statistics) among observers was excellent for the mosaic pattern (kappa: 0.76 for both the videoendoscopic inspection alone and dye staining) and the scalloped folds (kappa: 0.83 and 0.76, respectively) and was fair (kappa: 0.41 and 0.59, respectively) for the reduction in the number or loss of duodenal folds. This study confirms that videoduodenoscopy is useful in the detection of intestinal atrophy. Dye staining produces a better delineation of scalloped folds and mosaic pattern in the atrophic mucosa, but did not provide additional information to the expert endoscopist. Finally, interobserver agreement was excellent for the most prevalent signs.
the indication for duodenal ESD is highly controversial. EMR with a cap-fitted panendoscope (EMR-... more the indication for duodenal ESD is highly controversial. EMR with a cap-fitted panendoscope (EMR-C) is known as an effective treatment for esophageal tumors in particular. Recently, we have performed EMR-C as therapy for the superficial epithelial type of sporadic non-ampullary duodenal adenoma/carcinoma (SNADA) in our establishment. However, efficacy for duodenal tumors has not yet been reported. In this study, we assessed the efficacy of EMR-C for the superficial epithelial type of SNADA. Methods: We retrospectively evaluated 74 patients with SNADA who underwent endoscopic resection between May 2004 and October 2014 at our establishment. In addition, we assessed and compared the incidence rate of complications and frequency rate of en bloc resection according to the macroscopic appearance of the lesions and the methods of mucosal resection, especially of the superficial epithelial type of SNADA, between EMR-C and EMR. Results: The mean age of the patients was 63.7 12.0 years and 52 were male. Seventy-nine SNADA lesions were resected. The final pathological diagnoses, low-grade dysplasia, highgrade dysplasia, and intramucosal carcinoma were 21, 27, and 31, respectively. The mean size of the lesions was 10.5 5.8 mm. The numbers of macroscopic appearance of types Ip, Is, Is + IIa, IIa, IIa + IIc, and IIc according to the Paris endoscopic classification were 5, 9, 3, 27, 21, and 14, respectively. The numbers of lesions resected by polypectomy, EMR, EMR-C, strip biopsy, and ESD were 1, 33, 25, 9, and 11, respectively. The perforation rate with EMR and EMR-C were significantly lower than that with ESD (0% and 4.0% vs. 27.3%; p Z 0.002 and 0.041, chi-square test). For superficial epithelial lesions, the rate of en bloc resection in EMR-C was 96.0% (24 in 25 lesions), which was significantly higher than that in EMR (vs. 68.4%, 13 in 19 lesions; p Z 0.0139, chi-square test). Furthermore, no significant difference in the mean size of the resected superficial epithelial type of SNADA was observed between EMR-C and EMR (7.6 3.2 mm vs. 8.7 4.3 mm; p Z 0.361, MannWhitney U test). Conclusions: EMR-C had a significant higher rate of en bloc resection of the superficial epithelial type of SNADA than EMR. EMR-C is a safe and effective treatment of SNADA, especially of the superficial epithelial type.
BACKGROUND We recently demonstrated that the odds of contracting coronavirus disease 2019 (COVID-... more BACKGROUND We recently demonstrated that the odds of contracting coronavirus disease 2019 (COVID-19) in patients with celiac disease (CeD) is similar to that of the general population. However, how patients with CeD perceive their COVID-19 risk may differ from their actual risk. AIM To investigate risk perceptions of contracting COVID-19 in patients with CeD and determine the factors that may influence their perception. METHODS We distributed a survey throughout 10 countries between March and June 2020 and collected data on demographics, diet, COVID-19 testing, and risk perceptions of COVID-19 in patients with CeD. Participants were recruited through various celiac associations, clinic visits, and social media. Risk perception was assessed by asking individuals whether they believe patients with CeD are at an increased risk of contracting COVID-19 when compared to the general population. Logistic regression was used to determine the influencing factors associated with COVID-19 risk perception, such as age, sex, adherence to a gluten-free diet (GFD), and comorbidities such as cardiac conditions, respiratory conditions, and diabetes. Data was presented as adjusted odds ratios (aORs) RESULTS A total of 10737 participants with CeD completed the survey. From them, 6019 (56.1%) patients with CeD perceived they were at a higher risk or were unsure if they were at a higher risk of contracting COVID-19 compared to the non-CeD population. A greater proportion of patients with CeD perceived an increased risk of contracting COVID-19 when compared to infections in general due to their CeD (56.1% vs 26.7%, P < 0.0001). Consequently, 34.8% reported taking extra COVID-19 precautions as a result of their CeD. Members of celiac associations were less likely to perceive an increased risk of COVID-19 when compared to non-members (49.5% vs 57.4%, P < 0.0001). Older age (aOR: 0.99; 95%CI: 0.99 to 0.99, P < 0.001), male sex (aOR: 0.84; 95%CI: 0.76 to 0.93, P = 0.001), and strict adherence to a GFD (aOR: 0.89; 95%CI: 0.82 to 0.96, P = 0.007) were associated with a lower perception of COVID-19 risk and the presence of comorbidities was associated with a higher perception of COVID-19 risk (aOR: 1.38; 95%CI: 1.22 to 1.54, P < 0.001). CONCLUSION Overall, high levels of risk perceptions, such as those found in patients with CeD, may increase an individual’s pandemic-related stress and contribute to negative mental health consequences. Therefore, it is encouraged that public health officials maintain consistent communication with the public and healthcare providers with the celiac community. Future studies specifically evaluating mental health in CeD could help determine the consequences of increased risk perceptions in this population.
Nature Reviews Gastroenterology & Hepatology, Jan 3, 2022
Coeliac disease is a systemic disorder characterized by immune-mediated enteropathy, which is cau... more Coeliac disease is a systemic disorder characterized by immune-mediated enteropathy, which is caused by gluten ingestion in genetically susceptible individuals. The clinical presentation of coeliac disease is highly variable and ranges from malabsorption through solely extra-intestinal manifestations to asymptomatic. As a result, the majority of patients with coeliac disease remain undiagnosed, misdiagnosed or experience a substantial delay in diagnosis. Coeliac disease is diagnosed by a combination of serological findings of disease-related antibodies and histological evidence of villous abnormalities in duodenal biopsy samples. However, variability in histological grading and in the diagnostic performance of some commercially available serological tests remains unacceptably high and confirmatory assays are not readily available in many parts of the world. Currently, the only effective treatment for coeliac disease is a lifelong, strict, gluten-free diet. However, many barriers impede patients’ adherence to this diet, including lack of widespread availability, high cost, cross-contamination and its overall restrictive nature. Routine follow-up is necessary to ensure adherence to a gluten-free diet but considerable variation is evident in follow-up protocols and the optimal disease management strategy is not clear. However, these challenges in the diagnosis and management of coeliac disease suggest opportunities for future research. Despite advances in testing for coeliac disease over the past two decades, awareness of this disease remains suboptimal worldwide. Here, Makharia and colleagues summarize the current knowledge of epidemiology, risk factors, diagnosis and follow-up of coeliac disease and highlight opportunities for future research. Coeliac disease is a global disease with a worldwide prevalence of around 1%. Most patients with coeliac disease are undiagnosed, misdiagnosed and/or experience a substantial delay in diagnosis, which suggests an inadequate awareness of the spectrum of its clinical manifestations. Despite improvements in the diagnosis of coeliac disease, persistent challenges include high inter-assay and intra-assay variation in serological test performance and high inter-observer variability in the grading of villous abnormalities. The optimal follow-up strategy for coeliac disease is unclear and studies are needed to define the timing and role of serological testing, gluten immunogenic peptide measurement and repeat biopsy. Increased access to dietitians, improved tools to assess adherence to a gluten-free diet, increased availability of gluten-free foods and reduced gluten contamination of gluten-free food products are needed. As maintaining strict adherence to a gluten-free diet is restrictive and challenging, the development of adjunct or alternative non-dietary therapies for coeliac disease is crucial. Coeliac disease is a global disease with a worldwide prevalence of around 1%. Most patients with coeliac disease are undiagnosed, misdiagnosed and/or experience a substantial delay in diagnosis, which suggests an inadequate awareness of the spectrum of its clinical manifestations. Despite improvements in the diagnosis of coeliac disease, persistent challenges include high inter-assay and intra-assay variation in serological test performance and high inter-observer variability in the grading of villous abnormalities. The optimal follow-up strategy for coeliac disease is unclear and studies are needed to define the timing and role of serological testing, gluten immunogenic peptide measurement and repeat biopsy. Increased access to dietitians, improved tools to assess adherence to a gluten-free diet, increased availability of gluten-free foods and reduced gluten contamination of gluten-free food products are needed. As maintaining strict adherence to a gluten-free diet is restrictive and challenging, the development of adjunct or alternative non-dietary therapies for coeliac disease is crucial.
Serology has significantly revolutionized the knowledge of celiac disease (CD), leading to the id... more Serology has significantly revolutionized the knowledge of celiac disease (CD), leading to the identification of unsuspected patients in at-risk CD groups, thereby increasing the number of CD diagnoses compared to the pre-screening era. Several markers for CD with a progressive diagnostic accuracy have been identified over the years, but only three of them, i.e. anti-tissue transglutaminase (anti-tTG), anti-endomysial (EmA) and anti-deamidated gliadin antibodies (DGP) are currently assessed in the daily clinical practice. A thorough review of the literature identified 44 original studies published between 1998 to 2022 for a total of 5098 pediatric and adult CD patients (without selective IgA deficiency) and 11930 disease controls. The results highlighted that anti-tTG IgA exhibited a higher sensitivity for CD (93.4%) than EmA IgA (92.8%), DGP IgG (81.8%) and DGP IgA (83.8%). The specificity of EmA IgA (99%) resulted to be higher than those of anti-tTG IgA (95.8%), DGP IgG (96.4%) and DGP IgA (92.1%). In patients with selective IgA deficiency, a condition closely related to CD, serological screening should include one of the three antibodies of IgG class, since anti-tTG, DGP and EmA have a very similar diagnostic accuracy in this clinical setting. According to age, there are two main diagnostic strategies for CD detection. In children, the revised ESPGHAN 2020 guidelines established that CD could be diagnosed in both symptomatic and asymptomatic children by high anti-tTG IgA titers (>10 times the cut-off) and EmA positivity with no need to obtain duodenal biopsy and HLA typing. In adult patients, although high tTG IgA titers (confirmed by EmA IgA positivity) correlate with villous atrophy, an intestinal biopsy is still considered mandatory for confirming CD diagnosis. Currently, a case finding approach in at-risk groups is preferred to mass screening for CD detection.
In previous study in our laboratory, we had shown the effect of radiation on morphometry of small... more In previous study in our laboratory, we had shown the effect of radiation on morphometry of small bowel of rats after 7 days of a single external doses of 1,000 rads with liver protection. Our aim was to determine the effect of the same procedure of gamma radiation in rats on the morphometry and D-Xylose absorption 7, 14 and 21 days after injury and compared with a control group matched by weight. The D-Xylose absorption was measured by plasmatic D-Xylose, 60' after an intragastric doses of the pentose (0.5 grs/kg of body weight). Results: In the control group the mucosal thickness was 880 +/- 40 mu (X +/- SD), the vellous height was 640 +/- 37.6 mu and the xylosemia 15.91 +/- 11.39 mg%. At 7 days in the irradiated group (n: 11) the weight decreased 33.36 +/- 6.29% (Vs. control p less than .001), the mucosal thickness and the vellous height were 666 +/- 97 (p less than .001) and 466 +/- 66.9 mu (p less than .001) and the xylosemia 6.16 +/- 2.3 (p less than .05). The figures at 14 days (n: 16) showed improving and completed recovery at 21 days (n: 14). Irradiated rats were shown to be affected in weight, morphology and D-Xylose absorption, they began recovering after 7 days, and completed recovery at 21 days.
Background: Refractory sprue (RS) is a rare and severe celiac-like enteropathy not responding to ... more Background: Refractory sprue (RS) is a rare and severe celiac-like enteropathy not responding to a strict gluten-free diet. Although prognosis is generally poor, little is known about the long-term outcome of patients. Aim: to report baseline characteristics and long-term outcome of a series of patients diagnosed and treated in a single institution. Materials: We report a retrospective cohort of 25 consecutive patients (15 females; mean age 46 yr; range 28-71) diagnosed with RS based on the presence of a non-responsive celiac-like enteropathy. All patients were intensively treated with a gluten-free diet, steroids, nutritional support and immunosupression. Results: Clinical and biological characteristics of patients suggest that, at least, 24 patients had clear evidences of celiac disease. HLA DQ2/DQ8 genes were present in all the 24 patients typed and autoimmune enteropathy was excluded in all. According to the genotyping, 12 patients had a polyclonal lymphocyte population (RS type I) and 13 exhibited monoclonal TCR-gamma gene rearrangements (RS type II). Sixteen patients had evidence of ulcerative jejunitis (UJ) (7 in RS type I and 9 in type II). Overall median follow-up time after diagnosis of RS was 29 mo/patient (range 7 to 204) (45 mo for type I and 24 mo for type II). Overall mortality was 48% (12 patients), 6 in each type. Eight patients with UJ (50%), 3 with lymphoma (two T-cell and one B-cell type) and 4 (44%) without ulcers died during follow-up. The causes of death were sepsis in the context of a progressive deterioration but without overt malignancies (n=5), vascular causes (n=3) and severe malnutrition (n=1). Three- and 5-yr survival rate after diagnosis of RS for the overall population was 60% and 56%. There was no differences between type I (67%, 58%) and type II RS patients (54% for both periods). Patients with UJ had lower but non-significant 3- and 5-yr survival rates (56% and 50%, respectively) compared with patients without ulcers (78% and 66%). Survivors had a favorable outcome. While 11 patients persists asymptomatic, two other cases still have mild diarrhea and one low body weight. Conclusions: We confirm that RS is a severe celiac disease-related disorder with very high mortality. Diagnosis of overt lymphoma (12%) in our long-term follow-up was not as frequent as was reported by other groups. A proportion of patients persist in good health for a long time irrespective of the nature of the IEL infiltration or the presence of UJ.
The World Gastroenterology Organisation aims to guide health providers in the best management of ... more The World Gastroenterology Organisation aims to guide health providers in the best management of celiac disease by providing a concise document with recommendations based on the latest evidence and resulting from our global expert consensus process on best current practices. In this guideline, a review team consisting of experts from 4 continents provides an updating with concepts that have been developed since the publication of the earlier guideline (2013). The guideline explores the epidemiology of the disorder from a global perspective and updates the diagnostic work-up as well as therapeutic and follow-up aspects of celiac disease. As a novelty in celiac disease, the team also provides cascades that represent a hierarchical set of diagnostic and management options to deal with the disease, ranked by available resources. The aim in presenting cascades is to highlight appropriate, context-sensitive, and resource-sensitive management options for all geographic regions, regardless of whether they are considered as “developing,” “semideveloped,” or “developed.” World Gastroenterology Organisation cascades are context-sensitive, and the context is not necessarily defined solely by resource availability. As first-line tests for symptomatic and asymptomatic patients, the experts suggest antitissue transglutaminase (antitTG) immunoglobulin A (IgA)+total IgA. However, to confirm a positive initial test, or in case of borderline tissue transglutaminase (tTG) IgA titers, it is recommended that other tests should be added, such as IgA antiendomysium, which is strongly recommended in most other guidelines. Other immunoassays regarded as good tests include the antiendomysium antibody (EMA) and immunoglobulin G-deamidated gliadin peptide (IgG-DGP) tests. A combination of IgG-DGP and IgA-tTG is particularly useful for detecting patients with celiac disease who are IgA-deficient. IgG-DGP is reported to be able to detect a few more IgA-sufficient patients who are missed on IgA-transglutaminase (IgA-tTG) tests. For some authors, performing IgA-tTG+IgG-DGP—2 tests addressing different antigens—is better than if 2 tests addressing the same autoantigen (IgA-tTG+IgA-EMA) are carried out. There was intense debate in the review team regarding the most appropriate serological strategy for diagnosing celiac disease, and we present readers with all of the various strategies here.
The present study was designed to determine the diagnostic usefulness of videoduodenoscopic inspe... more The present study was designed to determine the diagnostic usefulness of videoduodenoscopic inspection alone and the addition of vital dye staining in the detection of celiac disease. We additionally sought to evaluate interobserver agreement for specific duodenoscopic markers of mucosal atrophy. One hundred sixty-seven consecutive subjects who underwent duodenoscopy for intestinal biopsy were included in a prospective controlled study. Endoscopic examination was performed by experienced endoscopists according to a set protocol using methylene blue (1%) dye. All procedures were recorded on videotape, but only 20 (10 with atrophy and 10 normal) were used in a blinded, independent, randomized analysis by five reviewers to evaluate interobserver agreement. Endoscopic signs indicative of mucosal atrophy were as follows: reduction in the number or loss of Kerkring&#39;s folds, &quot;scalloped&quot; folds, &quot;mosaic pattern,&quot; and visualization of the underlying blood vessels. Eighty-seven patients had celiac disease (57 newly diagnosed, 30 when treated). Seven treated patients had nonatrophic mucosa. In 80 patients the final diagnosis excluded celiac disease. Videoendoscopic inspection alone correctly identified 75 of 80 patients with complete mucosal atrophy and 86 of 87 with normal mucosa. False-negative diagnoses occurred in treated celiac patients with mild atrophy. Mosaic pattern (89%) and scalloped folds (86%) were the most useful endoscopic signs. Vital dye staining, as assessed by experienced endoscopists, provided identical results to those obtained by inspection alone. Sensitivity, specificity, and positive and negative predictive values for the presence of one or more than one feature were 94%, 100%, 100%, and 96%, respectively. The agreement (kappa statistics) among observers was excellent for the mosaic pattern (kappa: 0.76 for both the videoendoscopic inspection alone and dye staining) and the scalloped folds (kappa: 0.83 and 0.76, respectively) and was fair (kappa: 0.41 and 0.59, respectively) for the reduction in the number or loss of duodenal folds. This study confirms that videoduodenoscopy is useful in the detection of intestinal atrophy. Dye staining produces a better delineation of scalloped folds and mosaic pattern in the atrophic mucosa, but did not provide additional information to the expert endoscopist. Finally, interobserver agreement was excellent for the most prevalent signs.
the indication for duodenal ESD is highly controversial. EMR with a cap-fitted panendoscope (EMR-... more the indication for duodenal ESD is highly controversial. EMR with a cap-fitted panendoscope (EMR-C) is known as an effective treatment for esophageal tumors in particular. Recently, we have performed EMR-C as therapy for the superficial epithelial type of sporadic non-ampullary duodenal adenoma/carcinoma (SNADA) in our establishment. However, efficacy for duodenal tumors has not yet been reported. In this study, we assessed the efficacy of EMR-C for the superficial epithelial type of SNADA. Methods: We retrospectively evaluated 74 patients with SNADA who underwent endoscopic resection between May 2004 and October 2014 at our establishment. In addition, we assessed and compared the incidence rate of complications and frequency rate of en bloc resection according to the macroscopic appearance of the lesions and the methods of mucosal resection, especially of the superficial epithelial type of SNADA, between EMR-C and EMR. Results: The mean age of the patients was 63.7 12.0 years and 52 were male. Seventy-nine SNADA lesions were resected. The final pathological diagnoses, low-grade dysplasia, highgrade dysplasia, and intramucosal carcinoma were 21, 27, and 31, respectively. The mean size of the lesions was 10.5 5.8 mm. The numbers of macroscopic appearance of types Ip, Is, Is + IIa, IIa, IIa + IIc, and IIc according to the Paris endoscopic classification were 5, 9, 3, 27, 21, and 14, respectively. The numbers of lesions resected by polypectomy, EMR, EMR-C, strip biopsy, and ESD were 1, 33, 25, 9, and 11, respectively. The perforation rate with EMR and EMR-C were significantly lower than that with ESD (0% and 4.0% vs. 27.3%; p Z 0.002 and 0.041, chi-square test). For superficial epithelial lesions, the rate of en bloc resection in EMR-C was 96.0% (24 in 25 lesions), which was significantly higher than that in EMR (vs. 68.4%, 13 in 19 lesions; p Z 0.0139, chi-square test). Furthermore, no significant difference in the mean size of the resected superficial epithelial type of SNADA was observed between EMR-C and EMR (7.6 3.2 mm vs. 8.7 4.3 mm; p Z 0.361, MannWhitney U test). Conclusions: EMR-C had a significant higher rate of en bloc resection of the superficial epithelial type of SNADA than EMR. EMR-C is a safe and effective treatment of SNADA, especially of the superficial epithelial type.
BACKGROUND We recently demonstrated that the odds of contracting coronavirus disease 2019 (COVID-... more BACKGROUND We recently demonstrated that the odds of contracting coronavirus disease 2019 (COVID-19) in patients with celiac disease (CeD) is similar to that of the general population. However, how patients with CeD perceive their COVID-19 risk may differ from their actual risk. AIM To investigate risk perceptions of contracting COVID-19 in patients with CeD and determine the factors that may influence their perception. METHODS We distributed a survey throughout 10 countries between March and June 2020 and collected data on demographics, diet, COVID-19 testing, and risk perceptions of COVID-19 in patients with CeD. Participants were recruited through various celiac associations, clinic visits, and social media. Risk perception was assessed by asking individuals whether they believe patients with CeD are at an increased risk of contracting COVID-19 when compared to the general population. Logistic regression was used to determine the influencing factors associated with COVID-19 risk perception, such as age, sex, adherence to a gluten-free diet (GFD), and comorbidities such as cardiac conditions, respiratory conditions, and diabetes. Data was presented as adjusted odds ratios (aORs) RESULTS A total of 10737 participants with CeD completed the survey. From them, 6019 (56.1%) patients with CeD perceived they were at a higher risk or were unsure if they were at a higher risk of contracting COVID-19 compared to the non-CeD population. A greater proportion of patients with CeD perceived an increased risk of contracting COVID-19 when compared to infections in general due to their CeD (56.1% vs 26.7%, P < 0.0001). Consequently, 34.8% reported taking extra COVID-19 precautions as a result of their CeD. Members of celiac associations were less likely to perceive an increased risk of COVID-19 when compared to non-members (49.5% vs 57.4%, P < 0.0001). Older age (aOR: 0.99; 95%CI: 0.99 to 0.99, P < 0.001), male sex (aOR: 0.84; 95%CI: 0.76 to 0.93, P = 0.001), and strict adherence to a GFD (aOR: 0.89; 95%CI: 0.82 to 0.96, P = 0.007) were associated with a lower perception of COVID-19 risk and the presence of comorbidities was associated with a higher perception of COVID-19 risk (aOR: 1.38; 95%CI: 1.22 to 1.54, P < 0.001). CONCLUSION Overall, high levels of risk perceptions, such as those found in patients with CeD, may increase an individual’s pandemic-related stress and contribute to negative mental health consequences. Therefore, it is encouraged that public health officials maintain consistent communication with the public and healthcare providers with the celiac community. Future studies specifically evaluating mental health in CeD could help determine the consequences of increased risk perceptions in this population.
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Papers by Julio C Bai