Management of Patients with Refractory Coeliac Disease

Coeliac disease (CD) is an immune-mediated disorder affecting the small intestine. The condition represents an intolerance to gluten. Removal of dietary gluten permits recovery, with a full recovery for the majority of affected subjects. A percentage of affected subjects who do not improve with a gluten-free diet are considered to have refractory coeliac disease (RCD). Refractory coeliac disease is subdivided into type 1, characterised by a polyclonal expansion of intraepithelial lymphocytes (IELs) that have a normal phenotype, and type 2 (RCD2) which exhibits IELs with a monoclonal phenotype. Subjects with RCD carry a high risk of complications, including ulcerative jejunitis and lymphoma affecting the small intestine, the latter termed enteropathy-associated T-cell lymphoma (EATL).

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

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.

How to manage adult coeliac disease: perspective from the NHS England Rare Diseases Collaborative Network for Non-Responsive and Refractory Coeliac Disease

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.