title: Recognising and Managing Refractory Coeliac Disease: A Tertiary Centre Experience.
abstract: Refractory coeliac disease (RCD) is a rare complication of coeliac disease (CD) and involves malabsorption and villous atrophy despite adherence to a strict gluten-free diet (GFD) for at least 12 months in the absence of another cause. RCD is classified based on the T-cells in the intra-epithelial lymphocyte (IEL) morphology into type 1 with normal IEL and type 2 with aberrant IEL (clonal) by PCR (polymerase chain reaction) for T cell receptors (TCR) at the β/γ loci. RCD type 1 is managed with strict nutritional and pharmacological management. RCD type 2 can be complicated by ulcerative jejunitis or enteropathy associated lymphoma (EATL), the latter having a five-year mortality of 50%. Management options for RCD type 2 and response to treatment differs across centres and there have been debates over the best treatment option. Treatment options that have been used include azathioprine and steroids, methotrexate, cyclosporine, campath (an anti CD-52 monoclonal antibody), and cladribine or fluadribine with or without autologous stem cell transplantation. We present a tertiary centre's experience in the treatment of RCD type 2 where treatment with prednisolone and azathioprine was used, and our results show good response with histological recovery in 56.6% of treated individuals.
authors.affiliation: Gastroenterology Department, Guys and St Thomas' Hospital, Westminster Bridge Road, London SE1 7EH, UK. ikramnasr@hotmail.com.
authors.forename: Ikram
authors.initials: I
authors.lastname: Nasr
authors.affiliation: Allergy and Immunology Department, Pathology and Pharmacy Building, Royal London Hospital, 80 Newark Street, London E1 2ES, UK. drimannasr@gmail.com.
authors.forename: Iman
authors.initials: I
authors.lastname: Nasr
authors.affiliation: Department of Pathology, Guys and St Thomas' Hospital, Westminster Bridge Road, London SE1 7EH, UK. Carl.Beyers@viapath.co.uk.
authors.forename: Carl
authors.initials: C
authors.lastname: Beyers
authors.affiliation: Department of Pathology, Guys and St Thomas' Hospital, Westminster Bridge Road, London SE1 7EH, UK. fuju.chang@gstt.nhs.uk.
authors.forename: Fuju
authors.initials: F
authors.lastname: Chang
authors.affiliation: Gastroenterology Department, Guys and St Thomas' Hospital, Westminster Bridge Road, London SE1 7EH, UK. suzannedonnelly@nhs.uk.
authors.forename: Suzanne
authors.initials: S
authors.lastname: Donnelly
authors.affiliation: Gastroenterology Department, Guys and St Thomas' Hospital, Westminster Bridge Road, London SE1 7EH, UK. paul.ciclitira@kcl.ac.uk.
authors.forename: Paul J
authors.initials: PJ
authors.lastname: Ciclitira
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002446:Celiac Disease / Q000175:diagnosis* / Q000473:pathology / Q000628:therapy*; D055050:Diet, Gluten-Free; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D008286:Malabsorption Syndromes; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies
chemical_list: D007166:Immunosuppressive Agents
keywords: T-cell receptor (TCR); clonality; enteropathy associated T-cell lymphoma (EATL); gluten free diet (GFD); intra-epithelial cell lymphocytes (IEL); non-responsive coeliac disease (NRCD); polymerase chain reaction (PCR); refractory coeliac disease (RCD); ulcerative jejunitis; villous atrophy