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- Title
Recent advances in refractory coeliac disease: a review.
- Authors
Ho-Yen, C.; Chang, F.; van der Walt, J.; Mitchell, T.; Ciclitira, P.
- Abstract
Coeliac disease (CD) is an immune-mediated disease of the small intestine caused by intolerance to gluten. Removal of gluten from the diet results in a return to normal health for the majority of patients. A significant proportion of patients do not respond to a gluten-free diet and are considered to be suffering from refractory coeliac disease (RCD). Two types of RCD are now recognized: type 1 RCD is characterized by a polyclonal population of intraepithelial lymphocytes (IELs) with a normal immunophenotype, and type 2 RCD shows monoclonal IELs with an aberrant immunoprofile. Patients with RCD have a high risk of complications such as ulcerative jejunitis (UJ) and enteropathy-type T-cell lymphoma (ETTL). RCD2 may represent an early stage in the development of overt lymphoma. The diagnosis of RCD, therefore, has important implications, but remains a challenging area. In this paper we review the latest developments in RCD, including the diagnostic approach and a discussion of the key clinical, histological, immunohistochemical and molecular features of RCD and its complications.
- Subjects
CELIAC disease; MALABSORPTION syndromes; GLUTEN; LYMPHOMAS; SMALL intestine; LEUCOCYTES
- Publication
Histopathology, 2009, Vol 54, Issue 7, p783
- ISSN
0309-0167
- Publication type
Article
- DOI
10.1111/j.1365-2559.2008.03112.x