The Management of Complicated Celiac Disease

Department of Gastroenterology, VU University Medical Center, Amsterdam, The Netherlands.
Digestive Diseases (Impact Factor: 2.18). 09/2007; 25(3):230-6. DOI: 10.1159/000103891
Source: PubMed


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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    • "Type II RCD is associated with a risk of lymphoma and thus potentially a poor prognosis. Although no treatment has been found to be curative, chemotherapeutic agents are generally administered [24]. In severe cases, autologous stem cell transplantation has been employed for enteropathy associated T cell lymphoma [25]. "
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    ABSTRACT: Celiac disease can present in children and adults with a variety of manifestations including a rare complication known as ulcerative jejunitis, which is usually associated with refractory celiac disease in adults. The objective of this case report is to illustrate the first pediatric case of ulcerative jejunitis in celiac disease, diagnosed by capsule endoscopy, which is not associated with refractory celiac disease. The 9 year old girl presented with a history of abdominal pain and vomiting and a normal physical exam. Laboratory investigations revealed a mildly elevated IgA tissue transglutaminase antibody level and an IgA deficiency. Initial upper endoscopy was not conclusive for celiac disease. Further investigations included positive IgA anti-endomysium antibody, HLA DQ2 typing and a wireless capsule endoscopy consistent with delayed appearance of villi until the proximal to mid jejunum and mucosal ulcerations in the jejunum. Push enteroscopy with biopsies confirmed the diagnosis of celiac disease and ulcerative jejunitis. Immunohistochemical studies of the intraepithelial lymphocytes PCR amplification studies revealed oligoclonal T cell populations.. A repeat capsule study and upper endoscopy with biopsies, one year as well as 4 years following a strict gluten free diet showed normalization of the small bowel. Ulcerative jejunitis in association with celiac disease has never previously been described in children. Capsule endoscopy was essential to both the diagnosis of celiac disease and its associated ulcerative jejunitis. The repeat capsule endoscopy findings, one year following institution of a gluten free diet, also suggest that ulcerative jejunitis is not always associated with refractory celiac disease and does not necessarily dictate a poor outcome.
    BMC Gastroenterology 02/2014; 14(1):29. DOI:10.1186/1471-230X-14-29 · 2.37 Impact Factor
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    • "The most common symptoms in RCD include persistent diarrhoea, abdominal pain, and involuntary weight loss [13]. Moreover, fatigue, malaise, anaemia, hypoalbuminemia, vitamin deficiencies, and coexisting autoimmune disorders are frequently seen [13] [14]. The diagnosis RCDII becomes more likely when severe malnutrition , protein losing enteropathy, and ulcerative jejunitis are present [9]. "
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    ABSTRACT: A small subset of coeliac disease (CD) patients experiences persisting or recurring symptoms despite strict adherence to a gluten-free diet (GFD). When other causes of villous atrophy have been excluded, these patients are referred to as refractory celiac disease (RCD) patients. RCD can be divided in two types based on the absence (type I) or presence (type II) of an, usually clonal, intraepithelial lymphocyte population with aberrant phenotype. RCDI usually runs a benign course and may be difficult to be differentiated from uncomplicated, slow responding CD. In contrast, RCDII can be defined as low-grade intraepithelial lymphoma and frequently transforms into an aggressive enteropathy associated T-cell lymphoma with dismal prognosis. This paper describes the clinical characteristics of RCDI and RCDII, diagnostic approach, and the latest insights in treatment options.
    Gastroenterology Research and Practice 05/2013; 2013(1):518483. DOI:10.1155/2013/518483 · 1.75 Impact Factor
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    Solid-State Device Research Conference, 2005. ESSDERC 2005. Proceedings of 35th European; 10/2005
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