Azathioprine and prednisone combination therapy in refractory coeliac disease.
BACKGROUND
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.


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 TCRgamma-gene rearrangement and immunohistological changes as is currently reported in the literature. Therefore, immunophenotyping seems mandatory in the work-up of RCD.
Goerres
M S
MS
Department of Gastroenterology, Rijnstate Hospital, Arnhem, The Netherlands. mgoerres2@alysis.nl
Meijer
J W R
JW
Wahab
P J
PJ
Kerckhaert
J A M
JA
Groenen
P J T A
PJ
Van Krieken
J H J M
JH
Mulder
C J J
CJ
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D001379:Azathioprine / Q000627:therapeutic use*; D002446:Celiac Disease / Q000188:drug therapy*; D004359:Drug Therapy, Combination; D005260:Female; D019672:Genes, T-Cell Receptor; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011241:Prednisone / Q000627:therapeutic use*; D013601:T-Lymphocytes
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D001379:Azathioprine; D011241:Prednisone