Recognising and Managing Refractory Coeliac Disease: A Tertiary Centre Experience

Nutrients

Ikram Nasr¹  Iman Nasr²  Carl Beyers³  Fuju Chang³  Suzanne Donnelly¹ 

[1] Gastroenterology Department, Guys and St Thomas’ Hospital, Westminster Bridge Road, London SE1 7EH, UK;Allergy and Immunology Department, Pathology and Pharmacy Building, Royal London Hospital, 80 Newark Street, London E1 2ES, UK;Department of Pathology, Guys and St Thomas’ Hospital, Westminster Bridge Road, London SE1 7EH, UK;

关键词: non-responsive coeliac disease (NRCD);    refractory coeliac disease (RCD);    gluten free diet (GFD);    enteropathy associated T-cell lymphoma (EATL);    ulcerative jejunitis;    villous atrophy;    T-cell receptor (TCR);    clonality;    polymerase chain reaction (PCR);    intra-epithelial cell lymphocytes (IEL);   

DOI  :  10.3390/nu7125506

来源: mdpi

【 摘 要 】

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.

【 授权许可】

CC BY   
© 2015 by the authors; licensee MDPI, Basel, Switzerland.