Abstract
Coeliac disease (CD) can become primary or secondary refractory to a gluten free diet. Refractory coeliac disease refers to two distinct entities: Type I refractory coeliac disease (RCDI) which is hardly distinguishable from uncomplicated active coeliac disease and the malignant form, type II refractory coeliac disease (RCDII). Diagnosis is based on specialized small bowel investigations (upper endoscopy, enteroscopy, video capsule endoscopy) and techniques of intestinal intraepithelial lymphocytes analyses (immunohistochemistry, molecular biology, flow cytometry). Whereas survival of RCDI is close to that of CD, prognosis of RCDII is more undermined because of severe malnutrition and very high risk of overt lymphoma. Open capsule budesonide is currently the first line treatment. Nethertheless, recent advances in decephering pathogenic mechanisms of both types of RCD open new therapeutic strategies, to cure RCD and prevent Enteropathy Associated T cell Lymphoma.
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Malamut, G., Cerf-Bensussan, N. (2022). Introduction. In: Malamut, G., Cerf-Bensussan, N. (eds) Refractory Celiac Disease. Springer, Cham. https://doi.org/10.1007/978-3-030-90142-4_1
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DOI: https://doi.org/10.1007/978-3-030-90142-4_1
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