scholarly journals A Scoring System for Identifying Patients Likely to Be Diagnosed with Low-Grade Coeliac Enteropathy

Nutrients ◽  
2019 ◽  
Vol 11 (5) ◽  
pp. 1050 ◽  
Author(s):  
Fernando Fernández-Bañares ◽  
Anna Carrasco ◽  
Mercè Rosinach ◽  
Beatriz Arau ◽  
Roger García-Puig ◽  
...  
Keyword(s):  
Coeliac Disease ◽  
Scoring System ◽  
Villous Atrophy ◽  
Cell Receptor ◽  
Intraepithelial Lymphocytes ◽  
Low Grade ◽  
Positive Serology ◽  
Persistent Symptoms ◽  
Auc Value ◽  
Duodenal Biopsies

Background & Aims: Determining whether patients with lymphocytic enteritis (LE) have coeliac disease is a challenge. We analysed the variables associated with a low-grade coeliac enteropathy diagnosis in patients with suspected coeliac disease but without villous atrophy, and developed a scoring system to identify them. Methods: We collected data from 2010 through to 2016 on patients with lymphocytic enteritis and persistent symptoms compatible with the clinical spectrum of coeliac disease. One hundred and four patients starting on a gluten-free diet (GFD) were included. Duodenal biopsies were collected before the GFD and analysed for numbers of CD3+ T-cell receptor gamma delta+ (TCRγδ+), and CD3− intraepithelial lymphocytes. We performed a logistic regression analysis to identify factors associated with a low-grade coeliac enteropathy diagnosis. Results: Sixty-two patients achieved clinical remission after the GFD. Fifty of these 62 patients were diagnosed with low-grade coeliac enteropathy. Multivariate analysis identified the presence of >25% intraepithelial lymphocytosis, HLA-DQ2.5, positive serology, and increased numbers of TCRγδ+ cells with a low-grade coeliac enteropathy diagnosis. We developed a scoring system that identified patients with an area under the ROC curve (AUC) of 0.91. Scores of >10 had 86% sensitivity and 85% specificity. Conclusion: We developed a scoring system that identifies patients likely to be diagnosed with low-grade coeliac enteropathy with an AUC value of 0.91.

Diagnosis of coeliac disease by flow cytometry of intraepithelial lymphocytes: a new ‘gold’ standard?

2021 ◽  
pp. flgastro-2021-101838
Author(s):  
Kaninika Basu ◽  
Hannah Creasey ◽  
Nina Bruggemann ◽  
Jennifer Stevens ◽  
David Bloxham ◽  
...  
Keyword(s):  
Flow Cytometry ◽  
Coeliac Disease ◽  
Flow Cytometric Analysis ◽  
Cell Receptor ◽  
Intraepithelial Lymphocytes ◽  
Gluten Free Diet ◽  
Control Group ◽  
Gluten Free ◽  
Duodenal Biopsies

ObjectiveThe analysis of intraepithelial lymphocytes (IELs) by flow cytometry of duodenal biopsies—the ‘IEL’ lymphogram—has been proposed as a diagnostic test for coeliac disease. However, its clinical applicability has been limited due to variability in methods and definitions. This study set out to define useful parameters for the application of the IEL lymphogram to the diagnosis of coeliac disease.DesignFlow cytometry was performed on 117 sets of duodenal biopsies in 107 adult patients with active coeliac disease, long-term coeliac disease on a gluten free diet and a control group. The initial 95 samples were used for hypothesis generation for the subsequent samples comprising 12 patients with coeliac disease and 10 controls.ResultsRather than using single linear cut-offs for CD3 and T-cell receptor γδ (TCRγδ)+ve IELs, a discriminant function was identified as %CD3+ve IELs+2x(%TCRγδ+IELs)>100. This differentiated coeliac disease from control biopsies in the hypothesis generating group. These results were replicated in the validation group and found to be independent of histology in patients on long-term gluten free diet up to 12 years (combined sensitivity, 98.5%; specificity, 97.7%).ConclusionsFlow cytometric analysis of IELs is a highly sensitive and specific adjunct to serology and histological examination for the diagnosis of coeliac disease, even in individuals with coeliac disease following a gluten free diet who exhibit normal duodenal histology.

Refractory coeliac disease a reality: views from Pakistan

2016 ◽  
Vol 47 (1) ◽  
pp. 51-53
Author(s):  
Rajesh M Mandhwani ◽  
Rajesh K Wadhwa ◽  
Syed Mudassir Laeeq ◽  
Nasir Hasan Luck ◽  
Mohammad Mubarak ◽  
...  
Keyword(s):  
Coeliac Disease ◽  
Villous Atrophy ◽  
Immunosuppressive Agents ◽  
Signs And Symptoms ◽  
Intraepithelial Lymphocytes ◽  
Response To Treatment ◽  
Gluten Free ◽  
Refractory Celiac Disease ◽  
Small Intestinal ◽  
Refractory Coeliac Disease

Refractory coeliac disease (RCD) is described as persistence or recurrence of signs and symptoms of malabsorption with small-intestinal villous atrophy despite being on a strict gluten-free diet (GFD) for more than 12 months. RCD is a diagnosis of exclusion. There are two types of RCD, based upon the immunohistochemical features (presence of intraepithelial lymphocytes), response to treatment and prognosis. The treatment of RCD includes GFD and immunosuppressive agents. We hereby present a case of refractory celiac disease type II in a young man who later went on to develop Addisonian crisis and did not survive.

scholarly journals Coeliac Disease and Mast Cells

2019 ◽  
Vol 20 (14) ◽  
pp. 3400 ◽  
Author(s):  
Barbara Frossi ◽  
Marco De Carli ◽  
Antonino Calabrò
Keyword(s):  
T Cell ◽  
Mast Cells ◽  
Coeliac Disease ◽  
Cell Receptor ◽  
Intraepithelial Lymphocytes ◽  
Innate Immune ◽  
T Helper 1 ◽  
Inflammatory Microenvironment ◽  
Complex Interactions ◽  
Immunological Factors

Over the last decades, there has been an impressive progress in our understanding of coeliac disease pathogenesis and it has become clear that the disorder is the final result of complex interactions of environmental, genetic, and immunological factors. Coeliac disease is now considered a prototype of T-cell-mediated disease characterized by loss of tolerance to dietary gluten and the targeted killing of enterocytes by T-cell receptor αβ intraepithelial lymphocytes. Accumulating evidence, however, indicates that the induction of a gluten-specific T helper-1 response must be preceded by the activation of the innate immune system. Mast cells are key players of the innate immune response and contribute to the pathogenesis of a multitude of diseases. Here, we review the results of studies aimed at investigating the role of mast cells in the pathogenesis of coeliac disease, showing that these cells increase in number during the progression of the disease and contribute to define a pro-inflammatory microenvironment.

scholarly journals Update on the Diagnosis and Management of Refractory Coeliac Disease

2013 ◽  
Vol 2013 ◽  
pp. 1-9 ◽  
Author(s):  
Petula Nijeboer ◽  
Roy L. J. van Wanrooij ◽  
Greetje J. Tack ◽  
Chris J. J. Mulder ◽  
Gerd Bouma
Keyword(s):  
Coeliac Disease ◽  
Villous Atrophy ◽  
Treatment Options ◽  
Small Subset ◽  
Low Grade ◽  
Type I ◽  
Strict Adherence ◽  
Refractory Celiac Disease ◽  
Dismal Prognosis ◽  
Aberrant Phenotype

A small subset of coeliac disease (CD) patients experiences persisting or recurring symptoms despite strict adherence to a gluten-free diet (GFD). When other causes of villous atrophy have been excluded, these patients are referred to as refractory celiac disease (RCD) patients. RCD can be divided in two types based on the absence (type I) or presence (type II) of an, usually clonal, intraepithelial lymphocyte population with aberrant phenotype. RCDI usually runs a benign course and may be difficult to be differentiated from uncomplicated, slow responding CD. In contrast, RCDII can be defined as low-grade intraepithelial lymphoma and frequently transforms into an aggressive enteropathy associated T-cell lymphoma with dismal prognosis. This paper describes the clinical characteristics of RCDI and RCDII, diagnostic approach, and the latest insights in treatment options.

Symposium 1: Joint BAPEN and British Society of Gastroenterology Symposium on ‘Coeliac disease: basics and controversies’ Coeliac disease in the twenty-first century

2009 ◽  
Vol 68 (3) ◽  
pp. 234-241 ◽  
Author(s):  
William Dickey
Keyword(s):  
Coeliac Disease ◽  
Villous Atrophy ◽  
Tissue Transglutaminase ◽  
Disease Process ◽  
Histological Change ◽  
Intraepithelial Lymphocytes ◽  
British Society ◽  
Serological Testing ◽  
Multisystem Disorder ◽  
Number Of Patients

Coeliac disease (CD), traditionally perceived as a rare childhood condition presenting with malabsorption, is instead an autoimmune multisystem disorder usually presenting in adulthood, affecting ⩾1% of the population and linked to the genetic expression of human leucocyte antigens (HLA) DQ2 and DQ8. Presentation occurs most often in the 40–60 years age-group, but potentially at any age. Symptoms attributable to the gut or to malabsorption may be mild, non-specific or absent; under one-third of patients have diarrhoea and almost half are overweight. Histological diagnosis no longer requires small intestine villous atrophy. The Marsh classification recognizes increased intraepithelial lymphocytes and crypt hyperplasia with intact villi as part of the gluten enteropathy spectrum, while some individuals have more subtle abnormalities identified only on electron microscopy. Serological testing for CD autoantibodies (to endomysium and tissue transglutaminase) has revolutionized diagnosis, shifting the process towards primary care. However, a substantial number of patients with CD are seronegative, particularly those without villous atrophy. The autoantibody to endomysium may be produced before histological change. The immune response to transglutaminase is crucial to the disease process. An exciting new development is the link between antibodies to organ-specific transglutaminases and clinical presentation; transglutaminases 2 (gut), 3 (skin) and 6 (nervous system). Negative testing for CD does not preclude its development later and HLA testing may allow ‘once and for all’ exclusion. In conclusion, an increasing proportion of patients with CD do not meet the ‘classic’ picture of malabsorption, positive serological testing and villous atrophy. Insisting on all these criteria for diagnosis will result in under diagnosis.

scholarly journals Systematic Review and Meta-Analysis: Accuracy of Both Gamma Delta+ Intraepithelial Lymphocytes and Coeliac Lymphogram Evaluated by Flow Cytometry for Coeliac Disease Diagnosis

Nutrients ◽  
2019 ◽  
Vol 11 (9) ◽  
pp. 1992 ◽  
Author(s):  
Fernando Fernández-Bañares ◽  
Ana Carrasco ◽  
Albert Martín ◽  
Maria Esteve
Keyword(s):  
Flow Cytometry ◽  
Diagnostic Accuracy ◽  
Coeliac Disease ◽  
Meta Analysis ◽  
False Negative ◽  
Cell Receptor ◽  
Disease Diagnosis ◽  
Intraepithelial Lymphocytes ◽  
Significant Heterogeneity ◽  
Gamma Delta

It has been suggested that in doubtful cases of coeliac disease, a high CD3+ T-cell receptor gamma delta+ (TCRγδ+) intraepithelial lymphocyte count increases the likelihood of coeliac disease. Aim: To evaluate the diagnostic accuracy of both an isolated increase of TCRγδ+ cells and a coeliac lymphogram (increase of TCRγδ+ plus decrease of CD3− intraepithelial lymphocytes) evaluated by flow cytometry in the diagnosis of coeliac disease. Methods: The literature search was conducted in MEDLINE and EMBASE. The inclusion criteria were: an article that allows for the construction of a 2 × 2 table of true and false positive and true and false negative values. A diagnostic accuracy test meta-analysis was performed. Results: The search provided 49 relevant citations, of which 6 were selected for the analysis, which represented 519 patients and 440 controls. Coeliac lymphogram: The pooled S and Sp were 93% and 98%, without heterogeneity. The area under the SROC curve (AUC) was 0.98 (95% CI, 0.97–0.99). TCRγδ+: Pooled S and Sp were both 95%, with significant heterogeneity. The AUC was 0.97 (95% CI, 0.95–0.98). Conclusions: Both TCRγδ+ count and coeliac lymphogram assessed by flow cytometry in duodenal mucosal samples are associated with a high level of diagnostic accuracy for and against coeliac disease.

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