Presentation and long-term follow-up of refractory celiac disease: comparison of type I with type II.

Abstract

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.

• Authors: Georgia Malamut, Pauline Afchain, Virginie Verkarre, Thierry Lecomte, Aurélien Amiot, Diane Damotte, Yoram Bouhnik, Jean-Frédéric Colombel, Jean-Charles Delchier, Matthieu Allez, Jacques Cosnes, Anne Lavergne-Slove, Bertrand Meresse, Ludovic Trinquart, Elizabeth Macintyre, Isabelle Radford-Weiss, Olivier Hermine, Nicole Brousse, Nadine Cerf-Bensussan, Christophe Cellier
• Journal: Gastroenterology (Gastroenterology) Vol. 136 Issue 1 Pg. 81-90 (Jan 2009) ISSN: 1528-0012 [Electronic] United States
• PMID: 19014942 (Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't)

Chemical References

• Adrenal Cortex Hormones
• CD3 Complex
• CD8 Antigens

Topics

• Adrenal Cortex Hormones (therapeutic use)
• Adult
• CD3 Complex (analysis)
• CD8 Antigens (analysis)
• Celiac Disease (classification, drug therapy, immunology)
• Female
• Follow-Up Studies
• Humans
• Immunohistochemistry
• Male
• Middle Aged
• Survival Rate