Refractory Celiac Disease.

Georgia Malamut, Christophe Cellier

Journal: Gastroenterology clinics of North America 2019;48(1):137-144

PMID: 30711206

Full Text on Elsevier Science View on PubMed

Abstract

Refractory celiac disease (RCD) refers to persistence of malnutrition and intestinal villous 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 gastrointestinal and extraintestinal dissemination of the abnormal IELs, and high risk of overt lymphoma.

Address: Paris Descartes University, 15 rue de l'Ecole de Médecine, Paris 75006, France; Gastroenterology Department, Hôpital Européen Georges Pompidou, APHP, 20 rue Leblanc, Paris 75015, France; UMR1163 Institute Imagine, 24 Boulevard du Montparnasse, Paris 75015, France. Electronic address: [email protected].; Paris Descartes University, 15 rue de l'Ecole de Médecine, Paris 75006, France; Gastroenterology Department, Hôpital Européen Georges Pompidou, APHP, 20 rue Leblanc, Paris 75015, France; UMR1163 Institute Imagine, 24 Boulevard du Montparnasse, Paris 75015, France.

DOI: 10.1016/j.gtc.2018.09.010

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MeSH Terms: Budesonide, Celiac Disease, Endoscopy, Gastrointestinal, Flow Cytometry, Humans, Immunohistochemistry, Intestine, Small, Intraepithelial Lymphocytes, Malnutrition, Prognosis, Risk, Severity of Illness Index