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

International audienceBackground & Aims: Refractory celiac disease (RCD) was recently subdivided into 2 subtypes (RCD I and 11) 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 IT 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 m 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 TEL phenotype (P < .01), clonality (P = .01), and overt lymphoma. (P = .001) predicted short survival time. Only abnormal TEL 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;