title: Presentation and long-term follow-up of refractory celiac disease: comparison of type I with type II.
abstract: OBJECTIVE
  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.affiliation: Université Paris Descartes, Paris, France.
authors.forename: Georgia
authors.initials: G
authors.lastname: Malamut
authors.forename: Pauline
authors.initials: P
authors.lastname: Afchain
authors.forename: Virginie
authors.initials: V
authors.lastname: Verkarre
authors.forename: Thierry
authors.initials: T
authors.lastname: Lecomte
authors.forename: Aurélien
authors.initials: A
authors.lastname: Amiot
authors.forename: Diane
authors.initials: D
authors.lastname: Damotte
authors.forename: Yoram
authors.initials: Y
authors.lastname: Bouhnik
authors.forename: Jean-Frédéric
authors.initials: JF
authors.lastname: Colombel
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Delchier
authors.forename: Matthieu
authors.initials: M
authors.lastname: Allez
authors.forename: Jacques
authors.initials: J
authors.lastname: Cosnes
authors.forename: Anne
authors.initials: A
authors.lastname: Lavergne-Slove
authors.forename: Bertrand
authors.initials: B
authors.lastname: Meresse
authors.forename: Ludovic
authors.initials: L
authors.lastname: Trinquart
authors.forename: Elizabeth
authors.initials: E
authors.lastname: Macintyre
authors.forename: Isabelle
authors.initials: I
authors.lastname: Radford-Weiss
authors.forename: Olivier
authors.initials: O
authors.lastname: Hermine
authors.forename: Nicole
authors.initials: N
authors.lastname: Brousse
authors.forename: Nadine
authors.initials: N
authors.lastname: Cerf-Bensussan
authors.forename: Christophe
authors.initials: C
authors.lastname: Cellier
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000328:Adult; D017252:CD3 Complex / Q000032:analysis; D016827:CD8 Antigens / Q000032:analysis; D002446:Celiac Disease / Q000145:classification* / Q000188:drug therapy / Q000276:immunology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D015996:Survival Rate
chemical_list: D000305:Adrenal Cortex Hormones; D017252:CD3 Complex; D016827:CD8 Antigens