Refractory Celiac Disease

Refractory celiac disease (RCD) refers to persistence of malnutrition and intestinal villus atrophy for more than 1 to 2 years despite strict gluten-free diet in patients with celiac disease. Diagnosis remains difficult and impacts treatment and follow-up. RCD has been subdivided into 2 subgroups according to the normal (RCDI) or abnormal phenotype of intraepithelial lymphocytes (IELs) (RCDII). RCDII is considered as a low-grade intraepithelial lymphoma and has a poor prognosis due to gastroin-testinal and extraintestinal dissemination of the abnormal IELs, and high risk of overt lymphoma.