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Published on: December 15, 2011
The management of complicated celiac disease
A Al-toma1, W H M Verbeek, C J J Mulder
1Department of Gastroenterology, VU University Medical Center, Amsterdam, The Netherlands.
Insights
Refractory celiac disease (RCD) involves persistent symptoms despite a gluten-free diet (GFD). While RCD I may respond to immunosuppression, RCD II is often resistant, with cladribine and autologous stem cell transplantation (ASCT) showing promise for specific patient groups.
Area of Science:
- Gastroenterology
- Immunology
- Oncology
Background:
- Refractory celiac disease (RCD) is defined by persistent villous atrophy and symptoms despite a strict gluten-free diet (GFD) for over 12 months.
- RCD can be primary or secondary non-responsive to GFD, necessitating exclusion of other malabsorption causes and confirmation of celiac disease markers.
- RCD is classified into types, with RCD I potentially responding to immunosuppression, while RCD II is typically refractory to therapies.
Purpose of the Study:
- To review the diagnostic criteria and therapeutic approaches for refractory celiac disease.
- To evaluate the efficacy and safety of various treatments for RCD, including immunosuppressants, cladribine, and autologous stem cell transplantation (ASCT).
- To highlight the challenges in managing RCD, particularly RCD II, and the potential role of novel therapies.
Main Methods:
- Literature review of studies on refractory celiac disease diagnosis and treatment.
- Analysis of treatment outcomes for immunosuppressive therapy, cladribine, and ASCT in RCD patients.
- Comparison of treatment responses based on RCD type and aberrant T-cell presence.
Main Results:
- RCD I patients may benefit from immunosuppressive treatment.
- RCD II is generally resistant to medical therapies; corticosteroid response does not rule out enteropathy-associated T-cell lymphoma.
- Cladribine shows potential for reducing aberrant T cells and may offer long-term benefits in a subset of patients.
- ASCT demonstrates promising results in improving histology and clinical outcomes for patients with persistent high percentages of aberrant T cells, though its role in lymphoma prevention is unproven.
Conclusions:
- Management of RCD requires careful diagnosis, excluding other conditions, and considering specific treatment modalities based on RCD type.
- Immunosuppressive therapy, cladribine, and ASCT represent therapeutic options for refractory celiac disease, particularly for RCD II.
- Further research is needed to establish the long-term efficacy and role of ASCT in preventing lymphoma development in RCD patients.
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.
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