Introduction Refractory celiac disease (RCD) is a persistent malabsorption and villous atrophy despite adhering to a strict gluten-free diet (GFD) for at least 6–12 months in the absence of other cause 1. It is a rare complication of celiac disease (CD). RCD is classified based on the T-cells in the intraepithelial lymphocyte (IEL) morphology into type 1 with normal IEL and type 2 with aberrant IEL. RCD1 is managed with strict nutritional and pharmacological management. RCD2 can be complicated by ulcerative jejunitis or enteropathy associated lymphoma (EATL), the latter having a 5-year mortality of 8–20%. It is therefore necessary to investigate and manage RCD2 which has a less predicted response and has a poor prognosis due to the associated complications. Treatment options vary due to the low incidence of RCD2 and hence the small numbers of randomised control trials.
We present a single centre’s experience in the treatment of RCD2.
Methods We performed a single centre retrospective study of all cases of RCD2 using the celiac database in a single centre between 2000 and 2013. Case notes, biological and histological data were reviewed for patients with a diagnosis of RCD2 diagnosed between 2000 and 2013. All patients were treated with prednisolone, 20 mg, and azathioprine, 2 mg/kg/day with repeat small bowel biopsy and T cell receptor analysis by PCR at 4 monthly intervals.
Results Fourteen out of twenty patients with RCD2 were successfully treated with prednisolone and azathioprine to become either type 1 refractory celiac disease, in 12 patients, or celiac disease, in 2 patients, with a better 5-year survival. None of the type 2 refractory patients developed lymphoma on this treatment.
Conclusion Prednisolone combined with azathioprine can be used successfully to treat RCD2. Our experience shows it is a safe and successful approach to improve prognosis. We successfully treated 7 out of 10 patients with RCD2 with this regimen.
Reference 1 Alberto Rubio-Tapia, Joseph A Murray. Classification and Management of Refractory Celiac Disease. Gut 2010 April; 59(4):547–557
Disclosure of Interest None Declared.
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