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PTH-112 A Single Centre Experience Of Treatment Of Refractory Celiac Disease Type 2
  1. I Nasr1,1,
  2. S Donnelly1,
  3. C Ho-Yen2,
  4. T Mitchell3,
  5. F Chang2,
  6. P Ciclitira1
  1. 1Gastroenterology, Guy’s and St Thomas’ NHS Foundation Trust, London, UK
  2. 2Pathology, Guy’s and St Thomas’ NHS Foundation Trust, London, UK
  3. 3Molecular Diagnostic Services, Guy’s and St Thomas’ NHS Foundation Trust, London, UK

Abstract

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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