Behcet's disease (BD) is a recurrent systemic vasculitis of unknown et
iology. Genetic factors and infectious agents seem to be related ro th
e etiology and pathogenesis of the disease. ED is strongly associated
with HLA-B51 antigen in many ethnic groups. As there are differences i
n HLA profile in different ethnic groups, we designed this case-contro
l study to examine the association of HLA-B51 alleles and ED as well a
s to investigate the influence of sex, age at development of the Inter
national Study Group (ISG) for Behcet's Disease criteria and certain f
eatures of disease severity on the strength of this association.The st
udy includes 62 Greek ED patients who fulfil the ISG criteria for Behc
et's disease and 87 controls. Serological HLA Class-I typing was perfo
rmed by standard microlymphocytotoxicity technique. HLA-DNA typing for
the B5 group was performed in all B51 subjects and controls by PCR-SS
O. Allele B5101 was found in 80% of ED patients and in 26% of control
s (odds ratio (OR) 10.48, p < 10(-6)). Males who carry this allele hav
e a higher risk than females for ED (OR 16.97 and 5.74 respectively).
B5101 predisposes to ED at a younger age in both sexes and to the dev
elopment of erythema nodosum (OR = Il, P = 0.004). This was confirmed
by multiple logistic regression analysis. A weak but not significant a
ssociation was found between B5101 and uveitis (OR = 2). No associati
on was found between B5101 and vasculitis or skin lesions in either s
ex. It was concluded that in the Greek population allele B5101 is a p
redisposing marker for ED, as in most ethnic groups, and that this all
ele predisposes to the development of the disease at a younger age in
both sexes and to the development of erythema nodosum. (C) American So
ciety for Histocompatibility and Immunogenetics, 1998. Published by El
sevier Science Inc.