Clinical—Alimentary TractPresentation and Long-Term Follow-up of Refractory Celiac Disease: Comparison of Type I With Type II
Section snippets
Patients
The medical files of 83 consecutive patients with a diagnosis of RCD and referred to 6 large hospitals in France (Hospitals European Georges Pompidou, Lariboisière, Saint–Louis, Henri–Mondor, Claude Huriez, and Saint–Antoine) between 1992 and 2007 were reviewed retrospectively. Eleven patients were excluded because of other diagnoses: enteropathy associated with primary hypogammaglobulinemia (n = 4), collagenous sprue (n = 1), autoimmune enteropathy (n = 1), and lamina propria CD4+ T-cell or CD8
Clinical and biological data
Fourteen patients were diagnosed with RCD I (11 female and 3 male; mean age, 49.3 years) and 43 with RCD II (25 female and 18 male; mean age, 52.3 years) (Table 1). Primary resistance to GFD was observed in 5 patients (35.7%) with RCD I and in 21 patients (48.8%) with RCD II. In the remaining patients, the onset of a secondary resistance to GFD was observed after a mean of 10.2 (±7.6) years and 7.4 (±4.3) years in patients with RCD I and RCD II, respectively.
At diagnosis, all patients with RCD
Discussion
The present study of 57 patients with RCD confirms that they can be divided into 2 subtypes, I and II, according to the normal or aberrant phenotype of intestinal IELs. At diagnosis, malnutrition was more severe and lymphocytic gastritis and ulcerative jejunitis were more frequent in patients with RCD II than in patients with RCD I. The outcome of RCD II was much more severe, with a high mortality rate mainly due to the development of overt lymphoma. Our work confirms beyond doubt that RCD II
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N.C.-B. and C.C. contributed equally to this work.
The authors disclose the following: Supported by Lymphocoeliaque, Institut National du Cancer.