TY - JOUR
T1 - Primary biliary cirrhosis
T2 - Does X mark the spot?
AU - Selmi, Carlo
AU - Invernizzi, Pietro
AU - Miozzo, Monica
AU - Podda, Mauro
AU - Gershwin, M. Eric
PY - 2004/11
Y1 - 2004/11
N2 - Primary biliary cirrhosis (PBC) is an autoimmune disease of unknown etiology leading to progressive destruction of intrahepatic bile duct, with cholestasis, cirrhosis, and eventually liver failure. Epidemiological data indicate that environmental factors trigger autoimmunity in genetically susceptible individuals, although no definitive association of PBC with specific genes has been found. Further, no convincing explanation has been provided for the strong female predominance observed in the prevalence of PBC. However, we recently suggested that the enhanced monosomy X in peripheral white blood cells, and particularly in lymphocytes, of affected women might play a role in the induction of PBC. Such observations appear independent from the degree of cholestasis and specific for PBC. In this review we discuss the implications of these findings and their immunological implications.
AB - Primary biliary cirrhosis (PBC) is an autoimmune disease of unknown etiology leading to progressive destruction of intrahepatic bile duct, with cholestasis, cirrhosis, and eventually liver failure. Epidemiological data indicate that environmental factors trigger autoimmunity in genetically susceptible individuals, although no definitive association of PBC with specific genes has been found. Further, no convincing explanation has been provided for the strong female predominance observed in the prevalence of PBC. However, we recently suggested that the enhanced monosomy X in peripheral white blood cells, and particularly in lymphocytes, of affected women might play a role in the induction of PBC. Such observations appear independent from the degree of cholestasis and specific for PBC. In this review we discuss the implications of these findings and their immunological implications.
KW - Female precominance
KW - Monosomy X
KW - Primary biliary cirrhosis
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U2 - 10.1016/j.autrev.2004.05.003
DO - 10.1016/j.autrev.2004.05.003
M3 - Article
C2 - 15546796
AN - SCOPUS:8844239298
VL - 3
SP - 493
EP - 499
JO - Autoimmunity Reviews
JF - Autoimmunity Reviews
SN - 1568-9972
IS - 7-8 SPEC.ISS.
ER -