Treatment of Refractory Celiac Disease

A small subset of celiac disease (CD) patients is, or becomes, refractory to a gluten-free diet with persistent malabsorption and intestinal villous atrophy. Based on the presence or absence of a clonal expansion of premalignant intra-epithelial lymphocytes in the small intestinal epithelium patients are recognized as either Type I or Type II refractory celiac disease (RCD). While the former generally has a relatively mild disease course, Type II RCD is associated with a high risk of transformation towards an aggressive enteropathy associated T cell lymphoma. Since the first descriptions of RCD in the seventies, a large range of pharmacological approaches have been investigated for both types. In this Chapter, these approaches are extensively reviewed and a roadmap for future treatments is presented.