Presentation and long-term follow-up of refractory celiac disease: comparison of type I with type II.
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.
Malamut
Georgia
G
Université Paris Descartes, Paris, France.
Afchain
Pauline
P
Verkarre
Virginie
V
Lecomte
Thierry
T
Amiot
Aurélien
A
Damotte
Diane
D
Bouhnik
Yoram
Y
Colombel
Jean-Frédéric
JF
Delchier
Jean-Charles
JC
Allez
Matthieu
M
Cosnes
Jacques
J
Lavergne-Slove
Anne
A
Meresse
Bertrand
B
Trinquart
Ludovic
L
Macintyre
Elizabeth
E
Radford-Weiss
Isabelle
I
Hermine
Olivier
O
Brousse
Nicole
N
Cerf-Bensussan
Nadine
N
Cellier
Christophe
C
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
D000305:Adrenal Cortex Hormones; D017252:CD3 Complex; D016827:CD8 Antigens