Updated guidelines by the European Society for the Study of Coeliac Disease

In this issue of United European Gastroenterology Journal, ¹ the European Society for the Study of Coeliac Disease presents new recommendations for the management of coeliac disease and other gluten-related disorders. The guideline is comprehensive, and updated using the Grading of Recommendations, Assessment, Development and Evaluation (GRADE) system to provide strengths of recommendations and grades of supporting evidence. This guideline goes beyond coeliac disease, and includes definitions and approaches to treat other gluten-related disorders.

The guideline is consistent with prior recommendations of case finding and active testing to unmask coeliac disease, with the use of tissue transglutaminase antibodies as a first step in patients on a regular diet. The guideline highlights the increased risk of obtaining false positive results by using immunoglobulin (Ig)G-based serology in the absence of IgA deficiency. Accordingly, measurement of total IgA is also recommended. Point-of-care testing, and serology of saliva or stools, are not recommended. Multiple duodenal biopsies are recommended to confirm the diagnosis of coeliac disease in adults. This guideline supports European Society for Pediatric Gastroenterology, Hepatology, and Nutrition (ESPGHAN) criteria for non-biopsy diagnosis in children. ² Human Leukocyte Antigen (HLA)-DQ2/DQ8 testing is recommended in selected patients to rule out coeliac disease, or as initial step when on a gluten-free diet and diagnosis is questionable. A gluten-free diet is recognized as the only treatment for coeliac disease and advice by an expert dietitian is strongly recommended. Systematic follow-up is highlighted. The term ‘slow-responder’ is suggested for patients with persistent symptoms, signs and laboratory abnormalities despite at least 6–12 months of gluten-free diet. The term non-responsive is discouraged in view of clinical improvement over time in most ‘slow-responders’. Refractory coeliac disease is discussed in great detail. ³ T-cell flow cytometry is suggested as the most reliable method for classification. ⁴ Nutritional support is highlighted as essential therapy. Open-capsule budesonide is recommended as first-line therapy for refractory coeliac disease type 1. ⁵ Treatment for refractory coeliac disease type 2 is outlined, including the need to exclude the presence of enteropathy-type T-cell lymphoma, and the use of prednisone or open-capsule budesonide for ‘stable’ patients, and cladribine or fludarabine and autologous hematopoietic stem cell transplantation in ‘seriously ill’ patients. Referral to a clinical trial is highly recommended for patients with refractory coeliac disease type 2.

The second section deals with other gluten-related disorders. Non-coeliac gluten sensitivity continues to be a ‘diagnosis of exclusion’ after ruling out coeliac disease and wheat allergy. A multi-step diagnostic approach is recommended, with good characterization of baseline symptoms on a gluten-containing diet followed by a gluten-free diet trial of 6 weeks. Lack of symptomatic response excludes the diagnosis. A less-stringent gluten-free diet might be sufficient. Dermatitis herpetiformis requires skin biopsy confirmation. While a gluten-free diet is essential, dapsone is strongly recommended during the first 6–24 months. The term ‘refractory dermatitis herpetiformis’ is suggested for treated patients with healing of the intestine but active skin lesions. Gluten-free diet is suggested in patients with psoriasis and positive serology in the absence of other signs of coeliac disease. Screening is recommended in patients with dental enamel defects and recurrent oral aphthae. For the first time in a coeliac guideline, recommendations are provided to dentists on how to approach patients with suspicion of coeliac disease. A detailed description of potential neuro–psychiatric manifestations related to gluten is provided including gluten ataxia, peripheral neuropathy, gluten encephalopathy, temporal lobe epilepsy with hippocampal sclerosis, anxiety and depression. Increased awareness is recommended. We agree that neurological deficits may be common. ⁶

All evidence based guidelines are, by nature, backward looking. They contemplate what has gone before and often overweigh traditional practice and opinion. An acknowledgment of the accuracy of serology-based strategies in children is recognized, though the need for duodenal histology in all adults may be challenged by the very high positive predictive values of serology that meet certain criteria.^7,8 Recommendations from experts who carefully consider the evidence, often based on comprehensive studies, are in danger of further separating best practices from real life. Many patients are being diagnosed with coeliac disease on flimsy evidence and, even more troubling, advised to avoid gluten without any scientifically based evidence that they need to adapt to such a difficult lifestyle change. We have a responsibility to inform the general medical community of what is the best practice to protect patients from inappropriate treatments. The readers of this guideline should thank the authors for a superb synthesis and practical vision of current management, and the challenges of coeliac disease and other gluten-related disorders. It is the responsibility of the entire profession to share these recommendations widely. To do otherwise is to abdicate our responsibility to the field and to our patients.