Gastroenterology

Gastroenterology

Volume 136, Issue 1, January 2009, Pages 81-90
Gastroenterology

Clinical—Alimentary Tract
Presentation and Long-Term Follow-up of Refractory Celiac Disease: Comparison of Type I With Type II

https://doi.org/10.1053/j.gastro.2008.09.069Get rights and content

Background & Aims

Refractory celiac disease (RCD) was recently subdivided into 2 subtypes (RCD I and II) based on a normal or abnormal phenotype of intraepithelial lymphocytes (IELs), respectively. It is not clear, however, if these 2 entities differ in their presentation at diagnosis or long-term outcome. We compared the clinical and biological characteristics of RCD I and RCD II at diagnosis, the risk of developing an overt lymphoma, and the predictive factors of survival.

Methods

Medical files of 14 patients with RCD I and 43 with RCD II were analyzed retrospectively. Predictive factors of overt lymphoma and survival were studied in univariate and multivariate analyses.

Results

At diagnosis, malnutrition, ulcerative jejunitis, and lymphocytic gastritis were more common in patients with RCD II than RCD I (P < .05). Overt lymphomas occurred in 2 patients with RCD I and 16 with RCD II. In the univariate analysis, abnormal IEL phenotype and increased age at diagnosis of RCD were predictive factors for overt lymphoma. Abnormal IEL phenotype (P < .01), clonality (P = .01), and overt lymphoma (P = .001) predicted short survival time. Only abnormal IEL phenotype (P = .03) and overt lymphoma (P = .04) were predictive in the multivariate analysis. The 5-year survival rate was 93% in patients with RCD I and 44% with RCD II.

Conclusions

RCD II has a much more severe presentation and prognosis than patients with RCD I; <44% of patients with RCD II survive 5 years after diagnosis. Abnormal IEL phenotype is a predictive factor but not a necessary condition for the development of overt lymphoma.

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

References (26)

Cited by (0)

N.C.-B. and C.C. contributed equally to this work.

The authors disclose the following: Supported by Lymphocoeliaque, Institut National du Cancer.

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