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Clinical staging and survival in refractory celiac disease: a single center experience
Alberto Rubio-Tapia1, Darlene G Kelly, Brian D Lahr
1Division of Gastroenterology and Hepatology, Department of Medicine, Mayo Clinic College of Medicine, Rochester, MN, USA.
Insights
Refractory celiac disease (RCD) has high mortality, especially RCD II, often due to enteropathy-associated T-cell lymphoma (EATL). A new staging system may improve prognosis prediction for RCD patients.
Area of Science:
- Gastroenterology
- Immunology
- Oncology
Background:
- Refractory celiac disease (RCD) is characterized by persistent symptoms and intestinal damage despite a gluten-free diet.
- RCD presents with two immunophenotypes: normal/polyclonal intraepithelial lymphocytes (RCD I) or abnormal/monoclonal (RCD II).
Purpose of the Study:
- To describe clinical characteristics, treatment, and long-term outcomes in a large single-center cohort of RCD patients.
- To compare outcomes between RCD I and RCD II subtypes.
Main Methods:
- A cohort of 57 RCD patients (42 RCD I, 15 RCD II) was analyzed.
- Clinical characteristics and survival data were collected and compared between RCD subtypes.
Main Results:
- Overall 5-year survival was 70% (RCD I: 80%, RCD II: 45%).
- Mortality causes included refractory state and enteropathy-associated T-cell lymphoma (EATL).
- A novel staging system based on 5 prognostic factors showed significant differences in 5-year survival (Stage I: 96%, Stage II: 71%, Stage III: 19%).
Conclusions:
- RCD is associated with significant mortality, with RCD II posing a poorer prognosis due to EATL risk.
- A new prognostic staging model is proposed to enhance precision in predicting outcomes for RCD patients.
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.
