Celiac.com 01/20/2009 - Refractory celiac disease is a serious condition that occurs when celiac symptoms and intestinal damage continue even when the patient consumes a gluten-free diet.
There are two types of refractory celiac disease (RCD). In RCD type I, immuno-phenotype of intraepithelial lymphocytes (IELs) are normal and polyclonal, while RCD) type II, is noted for the presence of an abnormal intraepithelial lymphocyte (IEL) population (CD7+ CD3âˆ’ CD4/8-cytoplasmic CD3+). More than half of people with this condition develop enteropathy-associated T-cell lymphoma (EATL), a rare but virulent form of cancer with high mortality rates.
The team was made up of Wieke H.M. Verbeek, M.D., B. Mary E. von Blomberg, Ph.D., Petra E.T. Scholten, B.Sc., D. Joop Kuik, M.Sc., Chris J.J. Mulder, M.D. Ph.D., and Marco W.J. Schreurs, Ph.D., all from Amsterdam’s VU University Medical Center.
A certain type of IELs called TCRÎ³/Î´+ IELs may play an important role in repairing mucosa, maintaining homeostasis, and guarding against tumor development. TCRÎ³/Î´+ IELs in the human intestine have recently shown promise in the regulation of uncomplicated celiac disease.
In the study, the research team wanted to see if patients with RCD II had fewer TCRÎ³/Î´+ IELs than either RDC I, or celiac disease, an thus provide a possible explanation for ongoing mucosal damage and inflammation, and the development of abnormal T cells that tend to morph into EATL.
The team used a method called multi-parameter flow cytometric immuno-phenotyping on IELs obtained from recent small bowel biopsy specimens from a fairly large, distinct celiac disease and control groups (N = 87).
Patients with RCD II showed a much lower ratio of TCRÎ³ Î´+ IELs compared to either RCD I or celiac disease patients. Whereas, patients with uncomplicated celiac disease showed significantly higher numbers of TCRÎ³ Î´+ IELs than were found in the control group. The results showed the relationship between TCRÎ³ Î´+ IELs and aberrant IELs to be negative. It is interesting to note that TCRÎ³ Î´+ IELs numbers do rise in RCD II patients after effective treatment.
The negative relationship between TCRÎ³ Î´+ and abnormal IELs, together with their known role in regulating uncomplicated celiac disease, suggests that TCRÎ³ Î´+ IELs may play a crucial role in helping the body to repair mucosa, maintain homeostasis and possibly even guard against tumor development.
These cells may serve as important markers, along with the abnormal T cells, to help distinguish between types of celiac disease, and to gage the effectiveness of treatment efforts.
Am J Gastroenterol 2008;103:3152–3158