TY - JOUR
T1 - Presentation and Long-Term Follow-up of Refractory Celiac Disease
T2 - Comparison of Type I With Type II
AU - Malamut, Georgia
AU - Afchain, Pauline
AU - Verkarre, Virginie
AU - Lecomte, Thierry
AU - Amiot, Aurélien
AU - Damotte, Diane
AU - Bouhnik, Yoram
AU - Colombel, Jean Frédéric
AU - Delchier, Jean Charles
AU - Allez, Matthieu
AU - Cosnes, Jacques
AU - Lavergne-Slove, Anne
AU - Meresse, Bertrand
AU - Trinquart, Ludovic
AU - Macintyre, Elizabeth
AU - Radford-Weiss, Isabelle
AU - Hermine, Olivier
AU - Brousse, Nicole
AU - Cerf-Bensussan, Nadine
AU - Cellier, Christophe
N1 - Funding Information:
The authors disclose the following: Supported by Lymphocoeliaque, Institut National du Cancer.
PY - 2009/1
Y1 - 2009/1
N2 - 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.
AB - 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.
UR - https://www.scopus.com/pages/publications/59849109225
U2 - 10.1053/j.gastro.2008.09.069
DO - 10.1053/j.gastro.2008.09.069
M3 - Article
C2 - 19014942
AN - SCOPUS:59849109225
SN - 0016-5085
VL - 136
SP - 81
EP - 90
JO - Gastroenterology
JF - Gastroenterology
IS - 1
ER -