MR enteroclysis in refractory celiac disease: proposal and validation of a severity scoring system.

PURPOSE: To determine magnetic resonance (MR) enteroclysis findings in patients with uncomplicated celiac disease (CD), refractory CD (RCD) type I, and RCD type II, to develop and validate a scoring system to identify patients with RCD II and to determine the diagnostic accuracy of MR enteroclysis to detect CD-related malignancies.

MATERIALS AND METHODS: This study was performed with approval of the institutional review board. One radiologist blinded to clinical details retrospectively evaluated quantitative and qualitative criteria of 28 studies obtained in symptomatic patients with CD (uncomplicated CD, n = 10; RCD I, n = 8; RCD II, n = 10). A scoring system was developed by using parameters identified in multivariate analysis to be associated with RCD II, which two radiologists evaluated in a second group of 40 symptomatic patients with CD. Accuracy to detect malignancy was assessed in the total study group. Cumulative survival was evaluated in the total study group by using the Kaplan-Meier method.

RESULTS: MR enteroclysis could not be used to discriminate between uncomplicated CD and RCD I. The presence of less than 10 folds per 5 cm jejunum, mesenteric fat infiltration, and bowel wall thickening were associated with RCD II. A positive MR score was defined as the presence of two or more of these features. In the validation group, the MR score was positive in 13 of 15 patients with RCD II (sensitivity, 0.87) and negative in 24 of 25 patients without RCD II (specificity, 0.96). The 5-year survival rate was 95% in patients with a negative MR score and 56% in patients with a positive MR score (P < .0001). MR enteroclysis helped to identify the presence of seven of eight malignancies and to diagnose absence of malignancy in 58 of 60 studies.

CONCLUSION: MR enteroclysis can be used to investigate the presence of RCD II or malignancy in symptomatic patients with CD.