Tags

Type your tag names separated by a space and hit enter

Refractory coeliac disease.
Best Pract Res Clin Gastroenterol. 2005 Jun; 19(3):413-24.BP

Abstract

A small proportion of coeliac disease (CD) patients fail to improve after a gluten-free diet (GFD) and may be considered as atypical regarding their outcome (refractory coeliac disease). The aim of this study is to diagnose and manage patients with CD who fail to improve after a GFD. Refractory coeliac disease (RCD) is a malabsorption syndrome defined by persisting villous atrophy with, usually, an increase of intraepithelial lymphocytes (IELs) in the small bowel in spite of a strict GFD and comprises a heterogenous group of diseases. Some of these diseases have to be excluded and can be treated by specific therapies like antibiotics in tropical sprue and giardiasis and immune globulin substitution in common variable immunodeficiency, while other malabsorption syndromes are less well defined and may require immunosuppressive therapy. Standardized treatment, however, has not been evaluated in such patients so far. In a subgroup of patients with RCD, an abnormal intraepithelial lymphocyte (IEL) population may be observed with the lack of surface expression of usual T-cell markers (CD3-CD8 and/or the T-cell receptor (TCR)) on IELs associated with T-cell clonality pattern suggest the presence of an early enteropathy-associated T-cell lymphoma (EATL) in a subgroup of patients with RCD. This hypothesis has been supported by studies, which revealed progression into overt intestinal T-cell lymphomas in a subgroup of RCD. Steroid treatment has been reported effective even in patients with underlying early EATL. However, long-term results are unsatisfactory in most of these patients with RCD and parenteral nutrition has to be applied in some of these cases. First results with more aggressive chemotherapies and use of cytokines are under way. Due to the difficulty of diagnostic and therapeutic regimens patients should be referred to tertiary centres for coeliac disease.

Authors+Show Affiliations

Department of Medicine I, Gastroenterology, Infectious Diseases and Rheumatology, Charité, Campus Benjamin Franklin, Berlin, Germany. severin.daum@charite.deNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

15925846

Citation

Daum, Severin, et al. "Refractory Coeliac Disease." Best Practice & Research. Clinical Gastroenterology, vol. 19, no. 3, 2005, pp. 413-24.
Daum S, Cellier C, Mulder CJ. Refractory coeliac disease. Best Pract Res Clin Gastroenterol. 2005;19(3):413-24.
Daum, S., Cellier, C., & Mulder, C. J. (2005). Refractory coeliac disease. Best Practice & Research. Clinical Gastroenterology, 19(3), 413-24.
Daum S, Cellier C, Mulder CJ. Refractory Coeliac Disease. Best Pract Res Clin Gastroenterol. 2005;19(3):413-24. PubMed PMID: 15925846.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Refractory coeliac disease. AU - Daum,Severin, AU - Cellier,Christophe, AU - Mulder,Chris J J, PY - 2005/6/1/pubmed PY - 2005/12/16/medline PY - 2005/6/1/entrez SP - 413 EP - 24 JF - Best practice & research. Clinical gastroenterology JO - Best Pract Res Clin Gastroenterol VL - 19 IS - 3 N2 - A small proportion of coeliac disease (CD) patients fail to improve after a gluten-free diet (GFD) and may be considered as atypical regarding their outcome (refractory coeliac disease). The aim of this study is to diagnose and manage patients with CD who fail to improve after a GFD. Refractory coeliac disease (RCD) is a malabsorption syndrome defined by persisting villous atrophy with, usually, an increase of intraepithelial lymphocytes (IELs) in the small bowel in spite of a strict GFD and comprises a heterogenous group of diseases. Some of these diseases have to be excluded and can be treated by specific therapies like antibiotics in tropical sprue and giardiasis and immune globulin substitution in common variable immunodeficiency, while other malabsorption syndromes are less well defined and may require immunosuppressive therapy. Standardized treatment, however, has not been evaluated in such patients so far. In a subgroup of patients with RCD, an abnormal intraepithelial lymphocyte (IEL) population may be observed with the lack of surface expression of usual T-cell markers (CD3-CD8 and/or the T-cell receptor (TCR)) on IELs associated with T-cell clonality pattern suggest the presence of an early enteropathy-associated T-cell lymphoma (EATL) in a subgroup of patients with RCD. This hypothesis has been supported by studies, which revealed progression into overt intestinal T-cell lymphomas in a subgroup of RCD. Steroid treatment has been reported effective even in patients with underlying early EATL. However, long-term results are unsatisfactory in most of these patients with RCD and parenteral nutrition has to be applied in some of these cases. First results with more aggressive chemotherapies and use of cytokines are under way. Due to the difficulty of diagnostic and therapeutic regimens patients should be referred to tertiary centres for coeliac disease. SN - 1521-6918 UR - https://www.unboundmedicine.com/medline/citation/15925846/Refractory_coeliac_disease_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S1521-6918(05)00025-9 DB - PRIME DP - Unbound Medicine ER -