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Recognising and Managing Refractory Coeliac Disease: A Tertiary Centre Experience

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 l...

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Autores principales: Nasr, Ikram, Nasr, Iman, Beyers, Carl, Chang, Fuju, Donnelly, Suzanne, Ciclitira, Paul J.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4690058/
https://www.ncbi.nlm.nih.gov/pubmed/26633478
http://dx.doi.org/10.3390/nu7125506
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author Nasr, Ikram
Nasr, Iman
Beyers, Carl
Chang, Fuju
Donnelly, Suzanne
Ciclitira, Paul J.
author_facet Nasr, Ikram
Nasr, Iman
Beyers, Carl
Chang, Fuju
Donnelly, Suzanne
Ciclitira, Paul J.
author_sort Nasr, Ikram
collection PubMed
description 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.
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spelling pubmed-46900582015-12-30 Recognising and Managing Refractory Coeliac Disease: A Tertiary Centre Experience Nasr, Ikram Nasr, Iman Beyers, Carl Chang, Fuju Donnelly, Suzanne Ciclitira, Paul J. Nutrients Article 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. MDPI 2015-12-01 /pmc/articles/PMC4690058/ /pubmed/26633478 http://dx.doi.org/10.3390/nu7125506 Text en © 2015 by the authors; licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons by Attribution (CC-BY) license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Nasr, Ikram
Nasr, Iman
Beyers, Carl
Chang, Fuju
Donnelly, Suzanne
Ciclitira, Paul J.
Recognising and Managing Refractory Coeliac Disease: A Tertiary Centre Experience
title Recognising and Managing Refractory Coeliac Disease: A Tertiary Centre Experience
title_full Recognising and Managing Refractory Coeliac Disease: A Tertiary Centre Experience
title_fullStr Recognising and Managing Refractory Coeliac Disease: A Tertiary Centre Experience
title_full_unstemmed Recognising and Managing Refractory Coeliac Disease: A Tertiary Centre Experience
title_short Recognising and Managing Refractory Coeliac Disease: A Tertiary Centre Experience
title_sort recognising and managing refractory coeliac disease: a tertiary centre experience
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4690058/
https://www.ncbi.nlm.nih.gov/pubmed/26633478
http://dx.doi.org/10.3390/nu7125506
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