Refractory coeliac disease.
OBJECTIVE
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.


RESULTS
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.


CONCLUSIONS
Herein, we review here the distinctive diagnostic features of RCDI and RCDII, the risk of developing overt lymphoma and different therapeutic approaches.
Hôpital Européen Georges Pompidou APHP, Paris, France. georgia.malamut@egp.aphp.fr
Georgia
G
Malamut
Christophe
C
Cellier
D002446:Celiac Disease / Q000145:classification / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy; D055050:Diet, Gluten-Free / Q000009:adverse effects; D006801:Humans; D008223:Lymphoma / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D013274:Stomach Neoplasms / Q000175:diagnosis / Q000209:etiology / Q000628:therapy