CD is associated with two grades of disease linked precancerous states. This condition is known as refractory celiac disease (RCD), defined as malabsorption due to gluten-related enteropathy (villous atrophy or elevated intraepitheal lymphocytes) after initial or subsequent failure of a strict gluten-free diet (usually 1 year) and after exclusion of any disorder mimicking coeliac disease.
- RCD 1 involves precancerous tissues in which transformed T-cells continue to produce a response even though gluten is no longer present. Some RCD1 patients have been treated successfully with immunosuppressants (azathioprine, prednisone) when caught early. Elemental diet (proteins digested to amino acids) seems to be an effective alternative diet, indicating other proteins are stimulating the IEL. 5 year viability is high when treated. DQ representation is similar to non-RCD celiacs.
- RCD 2 involves neoplastic tissues that the lack of surface expression of usual T-cell markers.
- Increased expression of: Intracytoplasmic CD3e, Surface CD103
- Decreased expression of: CD8, CD4, TCR-alpha/beta
- Clonal T-cell expansion in RCD2 is not manageable with steroids (see: RCD 1) and sometimes manageable with chemotherapeutic drugs, however, more aggressive therapies seem more affective. A high percentage of RCD 2 patients spontaneously develop lymphoma (EATL), the 5 year survival rate is markedly lower than RCD1 but higher than lymphoma. DQ2.5/DQ2 individuals are more frequently found.
Causes of RCD.
- Coeliac disease
- Age at CD/GSE diagnosis - most people are over the age of 50 when first diagnosed at RCD
- Dietary nonconpliance - Some EATL appears years after diagnosis with non-GF diets.
- Length of latency - The length of time, often unknown in which the person is GSE+.
- Severity - The severity of the microscopic destruction appears to be a factor, and genetics appears now to play a role.
- Genetics - For RCD 2 and EATL, genetics plays large role DQ2.5/DQ2+ individuals are over-represented in the patient set
Read more about this topic: Gluten-sensitive Enteropathy Associated Conditions
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