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The management of complicated celiac disease.
Dig Dis. 2007; 25(3):230-6.DD

Abstract

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.

Authors+Show Affiliations

Department of Gastroenterology, VU University Medical Center, Amsterdam, The Netherlands.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

17827946

Citation

Al-toma, A, et al. "The Management of Complicated Celiac Disease." Digestive Diseases (Basel, Switzerland), vol. 25, no. 3, 2007, pp. 230-6.
Al-toma A, Verbeek WH, Mulder CJ. The management of complicated celiac disease. Dig Dis. 2007;25(3):230-6.
Al-toma, A., Verbeek, W. H., & Mulder, C. J. (2007). The management of complicated celiac disease. Digestive Diseases (Basel, Switzerland), 25(3), 230-6.
Al-toma A, Verbeek WH, Mulder CJ. The Management of Complicated Celiac Disease. Dig Dis. 2007;25(3):230-6. PubMed PMID: 17827946.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The management of complicated celiac disease. AU - Al-toma,A, AU - Verbeek,W H M, AU - Mulder,C J J, PY - 2007/9/11/pubmed PY - 2007/10/25/medline PY - 2007/9/11/entrez SP - 230 EP - 6 JF - Digestive diseases (Basel, Switzerland) JO - Dig Dis VL - 25 IS - 3 N2 - 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. SN - 1421-9875 UR - https://www.unboundmedicine.com/medline/citation/17827946/The_management_of_complicated_celiac_disease_ L2 - https://www.karger.com?DOI=10.1159/000103891 DB - PRIME DP - Unbound Medicine ER -