Semiquantitative reverse-transcriptase polymerase chain reaction (RT-P
CR) was used to study the T-cell. receptor (TCR) beta-chain variable (
V beta) region gene families expressed by T cells in 28 patients with
primary biliary cirrhosis (PBC), 20 normal controls, and 9 patients wi
th other chronic inflammatory hepatic diseases, We hypothesized that a
ctivation and clonal proliferation of T cells would lead to biases in
the T-cell repertoire of patients with PBC. Freshly harvested T cells
from both peripheral blood and liver tissue were examined for evidence
of biased V beta utilization. Individuals varied considerably in thei
r pattern of V beta expression, but several significant differences we
re noted in the PBC group. In peripheral blood, the mean level of V be
ta 6.1,3 expression was greater in PBC patients than in normal control
s. In the liver of PBC patients, the mean level of V beta 6.1,3 expres
sion was even higher than in the peripheral blood, indicating intrahep
atic accumulation of these T cells. The mean level of V beta 6.1,3 exp
ression was not significantly different in the blood compared with liv
er in patients with other Liver diseases, Expression of V beta 7 and V
beta 13.1 was also significantly greater in the liver than in the blo
od of PBC patients, but similar trends were also seen in the control l
iver group. These data show that specific alterations in the TCR reper
toire are present in the blood and liver of patients with PBC.