Relationship of HLA-DQ8 and severity of celiac disease: comparison of New York and Parisian cohorts.
OBJECTIVE
Celiac disease is a polygenic disorder associated with HLA-DQ2 or HLA-DQ8, which are present in greater than 90% of patients. The disease is considered milder in the United States compared with Europe. We assessed whether differences in the frequency of HLA type may account for differences in severity of the disease by using cohorts of patients from New York and Paris.


METHODS
HLA-DQ typing was performed on patients with celiac disease in New York and Paris. Clinical and pathologic data were compared between the New York and Parisian cohorts and also correlated with the different HLA types (HLA-DQ2, HLA-DQ2/-DQ8, HLA-DQ8).


RESULTS
Among these patients, the disease was milder in the New York cohort compared with the Parisian cohort. There were fewer patients with a classical presentation (45% and 89%, respectively; P < 0.001) and less severe pathology (total villous atrophy, 64% and 89%, respectively; P < 0.05), and less marked intraepithelial lymphocytosis (intraepithelial leukocytes [IELs]/100 enterocytes, 48.1 and 82.5, respectively; P < 0.0001). HLA-DQ2 homozygotes were less prevalent in the New York cohort compared with the Parisian cohort (59% and 79%, respectively; P = 0.08). HLA-DQ8 alleles were more prevalent in the New York cohort compared with the Parisian cohort (41% and 21%, respectively; P = 0.026). There was, however, no difference in the clinical or pathologic parameters of severity when we compared the groups based on HLA type.


CONCLUSIONS
HLA-DQ8 alleles were increased in the New York cohort of patients with celiac disease; however, this did not account for less severe manifestations of the disease.
Johnson
Timothy C
TC
Department of Medicine, Columbia University College of Physicians and Surgeons, New York, New York 10032, USA.
Diamond
Beverly
B
Memeo
Lorenzo
L
Negulescu
Horia
H
Hovhanissyan
Zara
Z
Verkarre
Virginie
V
Rotterdam
Heidrun
H
Fasano
Alessio
A
Caillat-Zucman
Sophie
S
Grosdidier
Etienne
E
Winchester
Robert
R
Cellier
Christophe
C
Jabri
Bana
B
Green
Peter H R
PH
D000328:Adult; D001284:Atrophy / Q000473:pathology; D016022:Case-Control Studies; D002446:Celiac Disease / Q000235:genetics* / Q000276:immunology* / Q000473:pathology; D003967:Diarrhea / Q000453:epidemiology / Q000209:etiology; D005006:Ethnicity; D005260:Female; D006683:HLA-DQ Antigens / Q000235:genetics*; D006720:Homozygote; D006801:Humans; D007413:Intestinal Mucosa / Q000166:cytology; D007421:Intestine, Small / Q000473:pathology; D008214:Lymphocytes / Q000378:metabolism; D008297:Male; D008871:Microvilli / Q000473:pathology; D008875:Middle Aged; D008938:Mitosis; D009519:New York City / Q000453:epidemiology; D010297:Paris / Q000453:epidemiology; D011446:Prospective Studies; D012720:Severity of Illness Index
D006683:HLA-DQ Antigens