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Refractory coeliac sprue is a diffuse gastrointestinal disease.
V Verkarre1, V Asnafi, T Lecomte
1Department of Pathology and Université René Descartes-Paris V (EA219), AP-HP, Hôpital Necker-Enfants Malades, 75015 Paris, France. virginie.verkarre@nck.ap-hop-paris.fr
Gut
|January 14, 2003
Summary
Refractory coeliac sprue (RCS) involves abnormal T cells spreading throughout the digestive tract and blood. This suggests RCS is a diffuse gastrointestinal disease, not just confined to the small intestine.
Area of Science:
- Gastroenterology
- Immunology
- Oncology
Background:
- Refractory coeliac sprue (RCS) is characterized by an aberrant clonal intraepithelial lymphocyte (IEL) population, considered a cryptic form of intestinal T cell lymphoma.
- Understanding the distribution of this abnormal IEL population is crucial for diagnosing and managing RCS.
Purpose of the Study:
- To investigate the distribution of the abnormal and monoclonal IEL population throughout the digestive tract in patients with RCS.
- To compare IEL phenotype and T cell clonality in gastric, colonic, and blood samples from RCS patients versus controls.
Main Methods:
- Compared frequency of lymphocytic gastritis (LG) and lymphocytic colitis (LC) in RCS, active coeliac disease (ACD), and gluten-free diet coeliac disease (GFD-CD) patients.
- Utilized immunohistochemistry and T cell receptor gamma gene (TCR-gamma) rearrangement analysis to assess IEL phenotype and clonality.
- Analyzed gastric, colonic, and blood samples from 15 RCS patients, 18 ACD patients, and 10 GFD-CD patients.
Main Results:
- Lymphocytic gastritis (LG) was found in 64% of RCS patients, and lymphocytic colitis (LC) in 55%.
- Aberrant IEL phenotypes were observed in LG and LC samples from RCS patients, unlike in coeliac disease patients.
- Monoclonal TCR-gamma rearrangements were detected in 62% of gastric, 80% of colonic, and 44% of blood samples from RCS patients.
Conclusions:
- The aberrant monoclonal IEL population in RCS disseminates beyond the small intestine to the blood and entire gastrointestinal epithelium.
- RCS should be considered a diffuse gastrointestinal disease due to the widespread nature of the abnormal IEL population.
- Findings highlight the systemic involvement in RCS, impacting diagnosis and treatment strategies.
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