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Recent advances in refractory coeliac disease: a review
Author(s) -
HoYen C,
Chang F,
Van Der Walt J,
Mitchell T,
Ciclitira P
Publication year - 2009
Publication title -
histopathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.626
H-Index - 124
eISSN - 1365-2559
pISSN - 0309-0167
DOI - 10.1111/j.1365-2559.2008.03112.x
Subject(s) - intraepithelial lymphocyte , coeliac disease , medicine , disease , immunophenotyping , enteropathy , lymphoma , population , immunology , refractory (planetary science) , pathology , gastroenterology , immune system , biology , antigen , environmental health , astrobiology
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.