Gastroenterology

Gastroenterology

Volume 136, Issue 1, January 2009, Pages 99-107
Gastroenterology

Clinical—Alimentary Tract
Clinical Staging and Survival in Refractory Celiac Disease: A Single Center Experience

https://doi.org/10.1053/j.gastro.2008.10.013Get rights and content

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.

Section snippets

Patients

The study group included patients with RCD treated at the Mayo Clinic Rochester between June 1998, when the first patient was included, and October 2007, the cutoff date for entry into this report. Most patients (>96%) were evaluated and treated in the Celiac Clinic (by J.A.M.).

Diagnostic Criteria for RCD

The internationally accepted criteria for classification of RCD (and subtypes) were used to maximize the correct allocation of patients by categories.4, 5, 6, 11 The operational definition of RCD case required major and

Patients

A total of 57 patients (67% female) with a median age at refractory state diagnosis of 59 years (range, 30–76 years) were included. Forty-two (74%; 29 female) and 15 (26%; 9 female) patients had RCD I and RCD II, respectively. Fifty-two (91%) patients were white, 3 were Hispanic, and 2 were African American. Forty-eight (84%) patients were evaluated for RCD because of persistent villous atrophy and the development of new symptoms or recurrence of diarrhea after initial clinical response to a

Discussion

The principal findings are (1) RCD I and RCD II are associated with high rates of mortality especially during the first 2 years after the diagnosis of the refractory state (5-year cumulative survival for the entire cohort, 70%) and (2) a new staging system for RCD is proposed based on the cumulative effect on survival of 5 prognostic factors scored at the time of the refractory state diagnosis: albumin ≤3.2 g/dL, hemoglobin ≤ 11 g/dL deciliter, age ≥65 years, the presence of aberrant

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The authors disclose the following: Supported by the National Institutes of Health (NIH) under Ruth L. Kirschstein National Research Service Award/Training Grant in Gastrointestinal Allergy and Immunology (T32 AI-07047) (to A.R.–T) and NIH grants DK-57892 and DK-070031 (to J.A.M).

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