Abstract
Concept of refractory coeliac disease (RCD) emerged in the past decade and refers to persistence of malnutrition and intestinal villous atrophy for more than 1 year strict gluten-free diet in coeliac patients. Diagnosis of this condition remains difficult and conditions treatment and follow-up. RCD has been subdivided into two subgroups according to the normal [type I RCD (RCDI)] or abnormal phenotype of intraepithelial lymphocytes [type II RCD (RCDII)]. RCDII is considered as a low-grade intraepithelial lymphoma and has a very poor prognosis, leading to intractable ulcerative jejunitis, gastrointestinal and extra-intestinal dissemination of the abnormal intraepithelial lymphocytes, and to their frequent transformation into a high-grade invasive lymphoma. Herein, we review here the distinctive diagnostic features of RCDI and RCDII, the risk of developing overt lymphoma and different therapeutic approaches.
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