Document Detail

The management of complicated celiac disease.
MedLine Citation:
PMID:  17827946     Owner:  NLM     Status:  MEDLINE    
Refractory celiac disease (RCD) is being defined as persisting or recurring villous atrophy with crypt hyperplasia and increased intraepithelial lymphocytes (IELs) in spite of a strict gluten-free diet (GFD) for >12 months or when severe persisting symptoms necessitate intervention independent of the duration of the GFD. RCD may not respond primarily or secondarily to GFD. All other causes of malabsorption must be excluded and additional features supporting the diagnosis of CD must be looked for, including the presence of antibodies in the untreated state and the presence of celiac-related HLA-DQ markers. In contrast to patients with a high percentage of aberrant T-cells, patients with RCD I seem to profit from an immunosuppressive treatment. RCD II is usually resistant to medical therapies. Response to corticosteroid treatment does not exclude underlying enteropathy-associated T-cell lymphoma. Cladribine seems to have a role, although it is less than optimal in the treatment of these patients. It may be considered, however, as the only treatment thus far studied that showed significant reduction of aberrant T cells, seems to be well tolerated, and may have beneficial long-term effects in a subgroup of patients showing significant reduction of the aberrant T-cell population. Autologous stem cell transplantation (ASCT) seems promising in those patients with persisting high percentages of aberrant T cells. The first group of patients treated with ASCT showed improvement in the small intestinal histology, together with an impressive clinical improvement. However, it remains to be proven if this therapy delays or prevents lymphoma development.
A Al-toma; W H M Verbeek; C J J Mulder
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Digestive diseases (Basel, Switzerland)     Volume:  25     ISSN:  1421-9875     ISO Abbreviation:  Dig Dis     Publication Date:  2007  
Date Detail:
Created Date:  2007-09-10     Completed Date:  2007-10-24     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8701186     Medline TA:  Dig Dis     Country:  Switzerland    
Other Details:
Languages:  eng     Pagination:  230-6     Citation Subset:  IM    
Copyright Information:
2007 S. Karger AG, Basel
Department of Gastroenterology, VU University Medical Center, Amsterdam, The Netherlands.
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MeSH Terms
Celiac Disease / complications,  diagnosis*,  therapy*
Diet Therapy
Endoscopy, Gastrointestinal
Gene Rearrangement
Genes, T-Cell Receptor gamma
HLA-DQ Antigens / blood
Immunosuppressive Agents / therapeutic use
Intestinal Mucosa / immunology
Intestine, Small / pathology
Lymphocytes / classification
Lymphoma, T-Cell / etiology
Peripheral Blood Stem Cell Transplantation
Transplantation, Autologous
Treatment Failure
Reg. No./Substance:
0/HLA-DQ Antigens; 0/Immunosuppressive Agents

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