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Review
. 2013 Sep;25(5):445-51.
doi: 10.1097/01.cco.0000432526.47228.b6.

Refractory coeliac disease

Georgia Malamut  1 , Christophe Cellier
Affiliations
Review

Refractory coeliac disease

Georgia Malamut et al. Curr Opin Oncol. 2013 Sep.

Abstract

Purpose of review: 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.

Recent findings: 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.

Summary: 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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