Refractory coeliac disease (RCD) is defined by persistent or recurrent malabsorptive symptoms and villous atrophy despite strict adherence to a gluten-free diet (GFD) for at least 6–12 months in the absence of other causes of non-responsive treated coeliac disease and overt malignancy. Symptoms are often severe and require additional therapeutic intervention besides a GFD. RCD can be classified as type 1 (normal intraepithelial lymphocyte phenotype), or type 2 (defined by the presence of abnormal (clonal) intraepithelial lymphocyte phenotype). Patients with RCD may never have responded to a GFD or may have relapsed despite adherence and initial response to the GFD. RCD type 1 usually improves after treatment with a combination of aggressive nutritional support, adherence to a GFD, and alternative pharmacological therapies. By contrast, clinical response to alternative therapies in RCD type 2 is less certain and the prognosis is poor. Severe complications such as ulcerative jejunitis and enteropathy-associated T cell lymphoma may occur in a subgroup of patients with RCD. The aims of this article are to (1) review recent advances in the diagnosis and management of patients with RCD, and (2) describe current and novel methods for classification of patients with RCD into categories that are useful to predict outcome and direct treatment.
- intraepithelial lymphocytes
- autologous hematopoietic stem cell transplantation
- coeliac disease
- enteropathy-associated T cell lymphoma
- endomysial antibodies
- gluten-free diet
- human leucocyte antigens
- positron emission tomography
- refractory coeliac disease
- tissue transglutaminase antibodies
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Funding This article was supported by the National Institutes of Health (NIH) under Ruth L. Kirschstein National Research Service Award/Training Grant in Gastrointestinal Allergy and Immunology (T32 AI-07047) (to AR-T) and the NIH grant DK-57892 (to JAM).
Competing interests Dr Murray is a consultant for ActogeniX Inc., Flamentera Inc., Ironwood Pharmaceuticals, and Alvine Inc. He is also an investigator for ALBA Therapeutics. Dr Rubio-Tapia declares no competing interest.
Provenance and peer review Not commissioned; externally peer reviewed
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