The management of complicated celiac disease.
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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ABSTRACT: Recent findings demonstrate the common genetic basis for many immune-mediated diseases, and consequently, the partially shared pathogenesis. We collected these findings and reviewed the extension of these overlaps to other disease characteristics. Two autoimmune diseases were selected that also share the specific target organ, the bowel. The etiology and immunopathogenesis of both conditions characterized by chronic intestinal inflammation, inflammatory bowel disease (IBD) and celiac disease (CeD), are not completely understood. Both are complex diseases with genetics and environment contributing to dysregulation of innate and adaptive immune responses, leading to chronic inflammation and disease. CeD constitutes a particular disease because the main environmental and genetic triggers are largely known. IBD comprises two main clinical forms, Crohn's disease and ulcerative colitis, which most likely involve a complex interplay between some components of the commensal microbiota and other environmental factors in their origin. These multifactorial diseases encompass a broad spectrum of clinical phenotypes and ages of onset, although the clinical presentation often differs depending on childhood or adult onset, with greater heterogeneity commonly observed in adults.World Journal of Gastroenterology 05/2014; 20(17):4846-4856. · 2.43 Impact Factor
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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. · 2.11 Impact Factor
- Zeitschrift fur Gastroenterologie. 07/2014; 52(7):711-743.