 |
Update on the Management of Refractory Coeliac
Disease
download
Full Article (PDF file)
Abdulbaqi Al-toma, Wieke HM Verbeek, Chris JJ Mulder
Department of Gastroenterology ,VU University Medical Center, Amsterdam,
The Netherlands
Abstract
True Refractory Coeliac Disease (RCD) is being currently defined
as persisting or recurring villous atrophy with crypt hyperplasia
and increased intraepithelial lymphocytes in spite of a strict gluten
free diet for more than 12 months or when severe persisting symptoms
necessitate intervention independent of the duration of the dietary
therapy. Currently two categories of RCD are being recognized: type
I without aberrant T-cells and type II with aberrant T-cells detected
by immunophenotyping by flowcytometric analysis or immunohistology
of the intestinal mucosa. Establishing the diagnosis of RCD requires
exclusion of other causes of villous atrophy and malignancies that
may complicate coeliac disease. In contrast to patients with a high
percentage of aberrant T cells, patients with RCD type I seem to
profit from an immunosuppressive treatment. In cases of RCD II with
persistent clinical symptoms and/or high percentage of aberrant
T cells in intestinal biopsies in spite of a corticosteroid treatment,
more aggressive therapeutic schemes should be considered. However,
no therapy seems to be curative in RCD II. Cladribine (2-CDA) seems
to have some role in the management of these patients. More recently,
high dose chemotherapy followed by autologous stem cell transplantation
has been used in patients resulting in a dramatic improvement in
the clinical, laboratory, histopathological and immunological parameters.
This review provides an overview of the currently available diagnostic
and therapeutic methods in a complicated form of coeliac disease.
Key words
Coeliac disease - refractory coeliac disease - pathogenesis - diagnosis
- therapy
|
|
 |
