TY - JOUR
T1 - Clinical Staging and Survival in Refractory Celiac Disease
T2 - A Single Center Experience
AU - Rubio-Tapia, Alberto
AU - Kelly, Darlene G.
AU - Lahr, Brian D.
AU - Dogan, Ahmet
AU - Wu, Tsung Teh
AU - Murray, Joseph A.
PY - 2009/1
Y1 - 2009/1
N2 - Background & Aims: Refractory celiac disease (RCD) occurs when both symptoms and intestinal damage persist or recur despite strict adherence to a gluten-free diet. In RCD, the immunophenotype of intraepithelial lymphocytes may be normal and polyclonal (RCD I) or abnormal and monoclonal (RCD II). The aim is to describe the clinical characteristics, treatment, and long-term outcome in a large single-center cohort of patients with RCD. Methods: We compared the clinical characteristics and outcome in 57 patients with RCD: 42 with RCD I and 15 with RCD II. Results: Fifteen of 57 patients died during follow-up (n = 8 with RCD I and n = 7 with RCD II), each within the first 2 years after RCD diagnosis. The overall 5-year cumulative survival is 70%, 80%, and 45% for the entire cohort, RCD I, and RCD II, respectively. The refractory state itself and enteropathy-associated T-cell lymphoma (EATL) were the most common causes of death, respectively. A new staging system is proposed based on the cumulative effect of 5 prognostic factors investigated at the time of the refractory state diagnosis: for patients in stages I, II, and III, the 5-year cumulative survival rate was 96%, 71%, and 19%, respectively (P < .0001). Conclusions: RCD is associated with high mortality with RCD II having an especially poor prognosis because of the development of EATL. A new staging model is proposed that may improve the precision of prognosis in patients with RCD.
AB - Background & Aims: Refractory celiac disease (RCD) occurs when both symptoms and intestinal damage persist or recur despite strict adherence to a gluten-free diet. In RCD, the immunophenotype of intraepithelial lymphocytes may be normal and polyclonal (RCD I) or abnormal and monoclonal (RCD II). The aim is to describe the clinical characteristics, treatment, and long-term outcome in a large single-center cohort of patients with RCD. Methods: We compared the clinical characteristics and outcome in 57 patients with RCD: 42 with RCD I and 15 with RCD II. Results: Fifteen of 57 patients died during follow-up (n = 8 with RCD I and n = 7 with RCD II), each within the first 2 years after RCD diagnosis. The overall 5-year cumulative survival is 70%, 80%, and 45% for the entire cohort, RCD I, and RCD II, respectively. The refractory state itself and enteropathy-associated T-cell lymphoma (EATL) were the most common causes of death, respectively. A new staging system is proposed based on the cumulative effect of 5 prognostic factors investigated at the time of the refractory state diagnosis: for patients in stages I, II, and III, the 5-year cumulative survival rate was 96%, 71%, and 19%, respectively (P < .0001). Conclusions: RCD is associated with high mortality with RCD II having an especially poor prognosis because of the development of EATL. A new staging model is proposed that may improve the precision of prognosis in patients with RCD.
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U2 - 10.1053/j.gastro.2008.10.013
DO - 10.1053/j.gastro.2008.10.013
M3 - Article
C2 - 18996383
AN - SCOPUS:59849098316
SN - 0016-5085
VL - 136
SP - 99
EP - 107
JO - Gastroenterology
JF - Gastroenterology
IS - 1
ER -