Celiac.com 01/30/2009 - Doctors from the Mayo clinic are proposing a new staging system for patients with Refractory Celiac Disease (RCD) after results of a recent study showed that their system offered greater accuracy and improved survival rates over the existing staging system. The new system will rely on the cumulative effect of 5 prognostic factors evaluated at the time of the refractory state diagnosis.
Refractory Celiac Disease occurs when both the symptoms and intestinal damage continue or recur, regardless of strict adherence to a gluten-free diet. In Refractory Celiac Disease, the immunophenotype of intraepithelial lymphocytes may be normal and polyclonal (RCD I) or abnormal and monoclonal (RCD II). The goal of this study was describe the clinical characteristics, treatment, and long-term outcome in a large single-center cohort of patients with RCD.
The team then applied the system to survival rates within the study: Stage I combined patients with a point score of 0 or 1 (n = 27), stage II patients with a point score of 2 or 3 (n = 16), and stage III patients with a point score of 4 (n = 14).
- Patients with total point scores of 0 (n = 15) or 1 (n = 12), showed overall 5-year survival rates of 93% and 100%, respectively.
- Patients with a score of 2 (n = 7) or 3 (n = 9) showed 5-year overall survival rates of 80% and 65%, respectively.
- Patients with a score of 4 (n = 10) or 5 (n = 4), the 5-year cumulative survival rates of 25% and 0%, respectively.
- For patients in stages I, II, and III, the 5-year cumulative survival rate was 96%, 71%, and 19%, respectively (P < .0001).
Refractory Celiac Disease generally carries a high rate of mortality, and the outcomes for RCD II have been especially poor because of the tendency for EATL to develop.
Citing the results, the team is proposing a new staging system based on the cumulative effect of 5 prognostic factors investigated at the time of the refractory state diagnosis
Gastroenterology 2009; 136:000–000