Aliment Pharmacol Ther 18(5):487-494, 2003.
M. S. Goerres*, J. W. R. Meijer, P. J. Wahab*, J. A. M. Kerckhaert, P. J. T. A. Groenen, J. H. J. M. Van Krieken, C. J. J. Mulder

Celiac.com 11/18/2003 - This very important Dutch study demonstrates a new and effective way of treating a subgroup of refractory celiac disease patients, those with normal intraepithelial T-lymphocytes (IELs). Considering the very poor outcome for those in the study with abnormal IELs (phenotypically immature intraepithelial T-lymphocytes defined by a lack of characteristic T-cell markers), we must hope that future research will soon yield an equally effective treatment. Here is the abstract of the study:

"Introduction: Refractory coeliac disease (RCD) is a rare syndrome with a poor prognosis, defined by malabsorption due to gluten-related enteropathy after initial or subsequent failure of a strict gluten-free diet and after exclusion of any disorder mimicking coeliac disease.

Patients and methods: Nineteen patients were included and treated. Based on intraepithelial T-lymphocyte(IEL) phenotyping, patients were recorded as having RCD type I with normal IELs, or RCD type II with phenotypically immature IELs defined by a lack of characteristic T-cell markers. Treatment consisted of azathioprine combined with prednisone for 1 year, which was tapered and, if possible, stopped.

Results: Clinical improvement was seen in nearly all patients in both groups. Eight of 10 RCD type I patients responded histologically, and complete normalization of villi was seen in four patients. In RCD type II, 6/8 patients developed enteropathy-associated T-cell lymphoma (EATL) and 7/8 patients died.

Conclusions: For the first time we report a promising therapeutic treatment option for RCD type I. In RCD type II, azathioprine and prednisone therapy (APT) is not effective, therefore we suggest that other (chemo)therapeutic agents are considered. Not all RCD type II patients presented with a monoclonal TCR?-gene rearrangement and immunohistological changes as is currently reported in the literature. Therefore, immunophenotyping seems mandatory in the work-up of RCD."

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