Enteropathies with villous atrophy but negative coeliac serology in adults: current issues

ObjectiveThe differential diagnosis and management of seronegative enteropathies is challenging due to the rarity of these conditions, the overlap of clinical and histopathological features and the current lack of an international consensus on their nomenclature.DesignThis is a narrative review providing pragmatic guide on the investigation and clinical management of seronegative enteropathies in adults based on the available literature and our clinical experience.ConclusionsSeronegative coeliac disease is the most frequent cause among the heterogeneous group of seronegative enteropathies and its diagnosis is confirmed by the clinical and histological response to a gluten-free diet after the exclusion of other causes of villous atrophy. Correct identification and targeted management of seronegative enteropathies is mandatory because of the variation in terms of clinical outcomes and prognosis.

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The Role of the Gluten-Free Diet in the Management of Seronegative Enteropathy

Nutrients ◽

10.3390/nu13114027 ◽

2021 ◽

Vol 13 (11) ◽

pp. 4027

Author(s):

Anna Szaflarska-Popławska

Keyword(s):

Differential Diagnosis ◽

Coeliac Disease ◽

Immunosuppressive Therapy ◽

Villous Atrophy ◽

Gluten Free Diet ◽

Evidence Based ◽

Gluten Free ◽

Clinical Challenge ◽

Immune Mediated

The differential diagnosis and treatment of seronegative enteropathy, also termed seronegative villous atrophy (SNVA), is a clinical challenge. Although seronegative coeliac disease (CD) is a frequent cause of SNVA, the aetiology can include immune-mediated, inflammatory, infectious, and drug-related forms. As a misdiagnosis of SNVA can result in patients being unnecessarily placed on a lifelong strict gluten-free diet or even given incorrect immunosuppressive therapy, the aim of this paper is to provide an evidence-based and practical approach for the workup and management of SNVA.

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Refractory coeliac disease a reality: views from Pakistan

Tropical Doctor ◽

10.1177/0049475516631710 ◽

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.

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A gluten-free diet score to evaluate dietary compliance in patients with coeliac disease

British Journal Of Nutrition ◽

10.1017/s0007114509301579 ◽

2009 ◽

Vol 102 (6) ◽

pp. 882-887 ◽

Cited By ~ 63

Author(s):

Federico Biagi ◽

Alida Andrealli ◽

Paola Ilaria Bianchi ◽

Alessandra Marchese ◽

Catherine Klersy ◽

...

Keyword(s):

Coeliac Disease ◽

Villous Atrophy ◽

Gluten Free Diet ◽

Gluten Free ◽

Efficient Tool ◽

Simple Method ◽

Dietary Compliance ◽

Endomysial Antibodies ◽

Diet Score ◽

Dietary Interview

A dietary interview performed by expert personnel is considered to be the most appropriate tool to check whether patients with coeliac disease follow a strict gluten-free diet. However, we currently have no straightforward and non-subjective method for performing such a dietary interview. We therefore developed a fast questionnaire based on four simple questions with a five-level score (0–IV). To verify whether our questionnaire is an efficient tool, we applied it to 168 coeliac patients (126 females and 42 males; mean age 42·4 (sd 12·9) years) on a gluten-free diet (median 82, 25th–75th percentile 50–108, range 15–389 months). The score we obtained was compared with the persistence of both villous atrophy and endomysial antibodies while on a gluten-free diet. A comparison with survival of the patients was also performed. Patients were interviewed over the phone by non-expert personnel. The questionnaire was completed in less than 1 min. The lowest results were significantly more frequent among the patients with a persistence of both villous atrophy and positive endomysial antibodies. Death risk was also significantly correlated with the lowest score results. We conclude that our questionnaire is a reliable and simple method of verifying compliance with a gluten-free diet.

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Symposium 1: Joint BAPEN and British Society of Gastroenterology Symposium on ‘Coeliac disease: basics and controversies’ Coeliac disease: optimising the management of patients with persisting symptoms?

Proceedings of The Nutrition Society ◽

10.1017/s0029665109001360 ◽

2009 ◽

Vol 68 (3) ◽

pp. 242-248 ◽

Cited By ~ 5

Author(s):

Kate E. Evans ◽

David S. Sanders

Keyword(s):

Coeliac Disease ◽

Management Strategy ◽

Villous Atrophy ◽

Management Plan ◽

Gluten Free Diet ◽

British Society ◽

Gluten Free ◽

Review Of The Literature ◽

Refractory Coeliac Disease ◽

Investigation Strategy

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.

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A score that verifies adherence to a gluten-free diet: a cross-sectional, multicentre validation in real clinical life

British Journal Of Nutrition ◽

10.1017/s0007114511007367 ◽

2012 ◽

Vol 108 (10) ◽

pp. 1884-1888 ◽

Cited By ~ 52

Author(s):

Federico Biagi ◽

Paola Ilaria Bianchi ◽

Alessandra Marchese ◽

Lucia Trotta ◽

Claudia Vattiato ◽

...

Keyword(s):

Coeliac Disease ◽

Villous Atrophy ◽

Gluten Free Diet ◽

Gluten Free ◽

Cross Sectional ◽

Simple Method ◽

Endomysial Antibodies ◽

Dietary Interview

A dietary interview performed by expert personnel is the best method to check whether patients with coeliac disease follow a strict gluten-free diet (GFD). We previously developed a score based on four fast and simple questions that can be administered even by non-expert personnel. The aim of the present study is to verify the reliability of our questionnaire in a new cohort of patients. The questionnaire has a five-level score. From March 2008 to January 2011, the questionnaire was administered to 141 coeliac patients on a GFD, who were undergoing re-evaluation. The score obtained was compared with persistence of both villous atrophy and endomysial antibodies (EMA). The rate of lower scores was higher among the patients with persistence of either villous atrophy (Fisher's exact, P < 0·001; test for trend, P < 0·001) or positive EMA (Fisher's exact, P = 0·001; test for trend, P = 0·018). Given that the coeliac patients have been well instructed on what a GFD means and on how to follow it, our questionnaire is a reliable and simple method to verify compliance to a GFD.

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Case of olmesartan-associated enteropathy and transient positive antitissue transglutaminase serology

BMJ Case Reports ◽

10.1136/bcr-2018-225518 ◽

2018 ◽

Vol 11 (1) ◽

pp. e225518 ◽

Cited By ~ 1

Author(s):

Jenan Ghaith ◽

Ismail A Raslan ◽

Andrew Szilagyi ◽

Mona Alameldin

Keyword(s):

Differential Diagnosis ◽

Small Bowel ◽

Coeliac Disease ◽

Villous Atrophy ◽

Transient Elevation

Olmesartan-associated enteropathy (OAE) is increasingly being recognised as a major differential diagnosis in patients with villous atrophy and negative coeliac disease (CD) serology. OAE and positive coeliac markers have rarely been reported. We report a case of diarrhoea and small bowel villous blunting associated with a transient elevation of antitissue transglutaminase antibody (ATTG). On discontinuation of olmesartan, symptoms improved, repeat biopsies were normal and levels of ATTG also returned normal. We discuss a possible explanation for the transient elevation in ATTG and the significance of considering OAE/CD overlap.

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Differential IL-13 Production by Small Intestinal Leukocytes in Active Coeliac Disease versus Refractory Coeliac Disease

Mediators of Inflammation ◽

10.1155/2013/939047 ◽

2013 ◽

Vol 2013 ◽

pp. 1-8 ◽

Cited By ~ 5

Author(s):

Sascha Gross ◽

Roy L. van Wanrooij ◽

Petula Nijeboer ◽

Kyra A. Gelderman ◽

Saskia A. G. M. Cillessen ◽

...

Keyword(s):

Coeliac Disease ◽

Potential Role ◽

Cytokine Production ◽

Villous Atrophy ◽

Gluten Free Diet ◽

Gluten Free ◽

Strict Adherence ◽

Aberrant Phenotype ◽

Small Intestinal ◽

Production Pattern

A small fraction of coeliac disease (CD) patients have persistent villous atrophy despite strict adherence to a gluten-free diet. Some of these refractory CD (RCD) patients develop a clonal expansion of lymphocytes with an aberrant phenotype, referred to as RCD type II (RCDII). Pathogenesis of active CD (ACD) has been shown to be related to gluten-specific immunity whereas the disease is no longer gluten driven in RCD. We therefore hypothesized that the immune response is differentially regulated by cytokines in ACD versus RCDII and investigated mucosal cytokine release after polyclonal stimulation of isolated mucosal lymphocytes. Secretion of theTH2cytokine IL-13 was significantly higher in lamina propria leukocytes (LPLs) isolated from RCDII patients as compared to LPL from ACD patients(P=0.05). In patients successfully treated with a gluten-free diet LPL-derived IL-13 production was also higher as compared to ACD patients(P=0.02). IL-13 secretion correlated with otherTH2as well asTH1cytokines but not with IL-10 secretion. Overall, the cytokine production pattern of LPL in RCDII showed more similarities with LPL isolated from GFD patients than from ACD patients. Our data suggest that different immunological processes are involved in RCDII and ACD with a potential role for IL-13.

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Active Coeliac: Disassembling Gluten and Coeliac Disease

Somatechnics ◽

10.3366/soma.2019.0279 ◽

2019 ◽

Vol 9 (2-3) ◽

pp. 188-205

Author(s):

Sofia Varino

Keyword(s):

Coeliac Disease ◽

Knowledge Production ◽

Food Additive ◽

Gluten Free ◽

Biomedical Knowledge ◽

Dietary Practices ◽

Object A ◽

Scientific Knowledge Production ◽

New Materialisms

This article follows the trajectories of gluten in the context of Coeliac disease as a gastrointestinal condition managed by lifelong adherence to a gluten-free diet. Oriented by the concept of gluten as an actant (Latour), I engage in an analysis of gluten as a participant in volatile relations of consumption, contact, and contamination across coeliac eating. I ask questions about biomedical knowledge production in the context of everyday dietary practices alongside two current scientific research projects developing gluten-degrading enzymes and gluten-free wheat crops. Following the new materialisms of theorists like Elizabeth A. Wilson, Jane Bennett, Donna Haraway and Bruno Latour, I approach gluten as an alloy, an impure object, a hybrid assemblage with self-organizing and disorganizing capacity, not entirely peptide chain nor food additive, not only allergen but also the chewy, sticky substance that gives pizza dough its elastic, malleable consistency. Tracing the trajectories of gluten, this article is a case study of the tricky, slippery capacity of matter to participate in processes of scientific knowledge production.

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