scholarly journals Non-Responsive Coeliac Disease: A Comprehensive Review from the NHS England National Centre for Refractory Coeliac Disease

Nutrients ◽  
2020 ◽  
Vol 12 (1) ◽  
pp. 216 ◽  
Author(s):  
Hugo A. Penny ◽  
Elisabeth M. R. Baggus ◽  
Anupam Rej ◽  
John A. Snowden ◽  
David S. Sanders

Coeliac disease is a common small intestinal enteropathy which manifests following ingestion of gluten in genetically susceptible individuals. Since gluten was identified as the driving factor in coeliac disease, the gluten-free diet (GFD) has remained the mainstay of treatment. While most individuals will display improvement in symptoms and signs of coeliac disease following institution of the GFD, up to 30% will continue to experience symptoms and/or have persisting intestinal inflammation. These individuals can be classified as having non-responsive coeliac disease (NRCD), which may be associated with dietary indiscretion, slow healing, refractory coeliac disease, and/or an alternative condition. The purpose of this review is to provide an overview of the causes of NRCD in adults, highlight a systematic approach to investigate these patients, and appraise the latest management aspects of this subset of coeliac disease.

2016 ◽  
Vol 47 (1) ◽  
pp. 51-53
Author(s):  
Rajesh M Mandhwani ◽  
Rajesh K Wadhwa ◽  
Syed Mudassir Laeeq ◽  
Nasir Hasan Luck ◽  
Mohammad Mubarak ◽  
...  

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.


2008 ◽  
Vol 61 (11) ◽  
pp. 1200-1202 ◽  
Author(s):  
U O’Shea ◽  
M Abuzakouk ◽  
C O’Morain ◽  
D O’Donoghue ◽  
K Sheahan ◽  
...  

Aims:Some patients with coeliac disease, despite strict adherence to a gluten-free diet, continue to have significant symptoms and/or a severe small intestinal histological lesion. The term “refractory coeliac disease” (rCD) is used to describe this condition. The purpose of this study was to investigate the value of tissue molecular markers reported to help in the diagnosis of rCD.Methods:Details on 61 patients with suspected rCD were collected. The clinical and laboratory findings in these patients were carefully evaluated, in part to determine whether patients were adhering to a strict gluten-free diet. The co-expression of CD3 and CD8 on intraepithelial lymphocytes was investigated by monoclonal antibody staining of small intestinal biopsy tissue; a finding of less than 50% CD3+ cells co-expressing CD8 was defined as an aberrant phenotype. T cell receptor gene rearrangement was assessed when a sufficient tissue sample was available.Results:A diagnosis of rCD was made in 38 patients based on clinical, laboratory and histological data. An aberrant intraepithelial lymphocyte population was found in 20 of these patients and in this group a clonal T cell population was found in five of seven patients tested. In the remaining 18 patients, the CD3/CD8 ratio was normal and two of seven tested had a clonal T cell population. After detailed monitoring, a diagnosis of rCD was excluded in the remaining 23 patients.Conclusions:This study supports the use of phenotypic and T cell clonality investigations in identifying patients with true rCD.


Biosensors ◽  
2019 ◽  
Vol 9 (2) ◽  
pp. 69 ◽  
Author(s):  
Maxine D. Rouvroye ◽  
Alfian Wicaksono ◽  
Sofie Bosch ◽  
Edo Savelkoul ◽  
James A. Covington ◽  
...  

Currently, the gold standard for diagnosis of coeliac disease (CD) is based on serology and gastroduodenoscopy with histology of duodenal mucosal biopsies. The aim of this study was to evaluate the potential of faecal volatile organic compounds (VOCs) analysis as a novel, non-invasive tool to discriminate between CD in remission in patients on a gluten-free diet (GFD), refractory coeliac disease (RCD) and controls without CD. Patients with an established diagnosis of CD on a GFD, RCD and healthy controls (HC) were instructed to collect a faecal sample. All subjects completed questionnaires on clinical symptoms, lifestyle and dietary information. Faecal VOCs were measured using gas chromatography-ion mobility spectrometry. A total of 13 CD, 7 RCD and 10 HC were included. A significant difference in VOC profiles between CD and RCD patients (area under the curve (AUC) ± 95% CI: 0.91 (0.79–1) p = 0.000) and between CD and HC (AUC ± 95% CI: 0.71 (0.51–0.91) p = 0.0254) was observed. We found no significant differences between faecal VOC patterns of HC and RCD. Based on faecal VOCs, CD could be discriminated from RCD and HC. This implies that faecal VOC analysis may hold potential as a novel non-invasive biomarker for RCD. Future studies should encompass a larger cohort to further investigate and validate this prior to application in clinical practice.


Nutrients ◽  
2020 ◽  
Vol 12 (11) ◽  
pp. 3540
Author(s):  
Paolo Usai-Satta ◽  
Mariantonia Lai

Celiac disease (CD) is a permanent, chronic, gluten-sensitive disorder characterized by small intestinal inflammation and malabsorption in genetically predisposed individuals [...]


2009 ◽  
Vol 68 (3) ◽  
pp. 242-248 ◽  
Author(s):  
Kate E. Evans ◽  
David S. Sanders

The vast majority of patients with coeliac disease will derive benefit from a gluten-free diet. However, some patients will not improve on the gluten-free diet or they will have a relapse of their symptoms. The present review will focus on this group of patients. Definitions for non-responsive coeliac disease and refractory coeliac disease will be provided. The most common reason for recurrent symptoms is continued gluten exposure. Other causes of persisting symptoms are discussed, including alternative causes of villous atrophy or co-existent pathology. Current literature is reviewed, including an initial investigation strategy for patients with persisting symptoms. A pragmatic management plan is described that can be initiated by any clinician. Finally, the current optimal investigational pathway for patients with refractory (or suspected refractory) coeliac disease is discussed and the reported effects of a number of therapeutic options are summarised. The aim of the present article is to provide clinicians with an up-to-date review of the literature in this clinical field and allow them to determine the most appropriate management strategy.


Author(s):  
Elisabeth MR Baggus ◽  
Anupam Rej ◽  
Annalisa Schiepatti ◽  
Lauren J Marks ◽  
Nick Trott ◽  
...  

2017 ◽  
Vol 64 (1) ◽  
pp. 25-33
Author(s):  
Dan Olteanu ◽  
◽  
Alexandru Diaconescu ◽  
Radu Voiosu ◽  
Andrei Voiosu ◽  
...  

Coeliac disease incidence rised during the last 50 years and represents a concern by diagnostic problems and costs. The recent data regarding etiology, pathogeny, comparative diagnostic value of serology and small intestinal biopsy are summarised. The new data about refractory celiac disease to gluten free diet and therapeutic perspectives are also presented (glutenases, larazotide acetate, genetic alteration of cereals, tissulary transglutaminase inhibitors etc).


2016 ◽  
Vol 63 (4) ◽  
pp. 272-279
Author(s):  
Dan Olteanu ◽  
◽  
Alexandru Diaconescu ◽  
Radu Voiosu ◽  
Andrei Voiosu ◽  
...  

Coeliac disease incidence raised during the last 50 years and represents a concern by diagnostic problems and costs. The recent data regarding etiology, pathogeny, comparative diagnostic value of serology and small intestinal biopsy are summarised. New data about refractory celiac disease to gluten free diet and therapeutic perspectives are also presented (glutenases, larazotide acetate, genetic alteration of cereals, tissulary transglutaminase inhibitors etc).


Nutrients ◽  
2020 ◽  
Vol 12 (2) ◽  
pp. 467
Author(s):  
Hanna Sankari ◽  
Minna Hietikko ◽  
Kalle Kurppa ◽  
Katri Kaukinen ◽  
Eriika Mansikka ◽  
...  

Dermatitis herpetiformis (DH), a cutaneous manifestation of coeliac disease, is characterized by transglutaminase (TG) 3-targeted dermal immunoglobulin A (IgA) deposits. The treatment for DH is the same as for coeliac disease, namely a life-long gluten-free diet. DH patients typically have gluten-dependent circulating autoantibodies targeting TG3 and TG2, and plasma cells secreting such autoantibodies have been detected in the small intestinal mucosa. This study investigates the gluten-responsiveness of intestinal TG3 and TG2 antibody-secreting plasma cells in 16 treated DH patients undergoing a gluten challenge. The frequency of both plasma cell populations increased significantly during the challenge, and their frequency correlated with the corresponding serum autoantibody levels at post-challenge. TG3-specific plasma cells were absent in all 18 untreated coeliac disease patients and seven non-coeliac control subjects on gluten-containing diets. These findings indicate that, in DH, both intestinal TG3- and TG2-antibody secreting plasma cells are gluten-dependent, and that TG3-antibody secreting plasma cells are DH-specific.


2019 ◽  
Vol 11 (3) ◽  
pp. 235-242 ◽  
Author(s):  
Elisabeth Megan Rose Baggus ◽  
Marios Hadjivassiliou ◽  
Simon Cross ◽  
Hugo Penny ◽  
Heidi Urwin ◽  
...  

Adult coeliac disease (CD) affects approximately 1% of the population. Most patients diagnosed will respond to a gluten-free diet; however, up to 30% may have persisting symptoms. Such patients may have ongoing issues associated with adherence, non-responsive CD or refractory CD. This article provides a clinical overview of how to manage this group of patients with persisting symptoms, including an investigational algorithm and details of how to contact the National Health Service England Rare Diseases Collaborative Network for Non-Responsive and Refractory Coeliac Disease. We hope this will be a valuable source of contemporary information for all UK gastroenterologists and internationally.


Sign in / Sign up

Export Citation Format

Share Document