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Refractory Celiac Disease: What the Gastroenterologist Should Know

Overview
Journal Int J Mol Sci
Publisher MDPI
Date 2024 Oct 16
PMID 39408713
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Abstract

Fewer than 1% of patients with celiac disease (CD) will develop refractory CD (RCD). As such, most gastroenterologists might never need to manage patients with RCD. However, all gastroenterologists must be familiarized with the basic concepts of RCD and non-responsive CD (NRCD), since it can present as a severe disease with high mortality, not only due to intestinal failure, but also due to progression to enteropathy-associated T cell lymphoma (EATL) and a higher susceptibility to life-threatening infections. The diagnostic workup and differential diagnosis with other causes of gastrointestinal symptoms and villous atrophy, as well as the differentiation between type I and II RCD, are complex, and may require specialized laboratories and reference hospitals. Immunosuppression is efficient in the milder RCDI; however, the treatment of RCDII falls short, with current options probably only providing transient clinical improvement and delaying EATL development. This review summarizes the current diagnostic and therapeutic approach for patients with RCD that all doctors that manage patients with CD should know.

References
1.
Al-Toma A, Volta U, Auricchio R, Castillejo G, Sanders D, Cellier C . European Society for the Study of Coeliac Disease (ESsCD) guideline for coeliac disease and other gluten-related disorders. United European Gastroenterol J. 2019; 7(5):583-613. PMC: 6545713. DOI: 10.1177/2050640619844125. View

2.
Hussein S, Gindin T, Lagana S, Arguelles-Grande C, Krishnareddy S, Alobeid B . Clonal T cell receptor gene rearrangements in coeliac disease: implications for diagnosing refractory coeliac disease. J Clin Pathol. 2018; 71(9):825-831. DOI: 10.1136/jclinpath-2018-205023. View

3.
Nijeboer P, van Wanrooij R, van Gils T, Wierdsma N, Tack G, Witte B . Lymphoma development and survival in refractory coeliac disease type II: Histological response as prognostic factor. United European Gastroenterol J. 2017; 5(2):208-217. PMC: 5349358. DOI: 10.1177/2050640616646529. View

4.
Al-Toma A, Visser O, van Roessel H, von Blomberg B, Verbeek W, Scholten P . Autologous hematopoietic stem cell transplantation in refractory celiac disease with aberrant T cells. Blood. 2006; 109(5):2243-9. DOI: 10.1182/blood-2006-08-042820. View

5.
Therrien A, Silvester J, Leonard M, Leffler D, Fasano A, Kelly C . Enteric-Release Budesonide May Be Useful in the Management of Non-Responsive Celiac Disease. Dig Dis Sci. 2020; 66(6):1989-1997. PMC: 8515997. DOI: 10.1007/s10620-020-06454-5. View