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Primary Biliary Cirrhosis: Pathology and Pathogenesis, pp. 109-144 $100.00
Authors:  (Yasuni Nakanuma, Yasunori Sato, Maylee Hsu, Hiroko Ikeda, Motoko Saski, Kenichi Harada, Kanazawa University Graduate School of Medicine, Kanazawa, Japan, and others)
Primary biliary cirrhosis (PBC) is an autoimmune liver disease which mainly affects
middle to old aged females. Clincially, PBC is a chronic cholestatic liver disease, and the
term PBC was established in 1950. Since then, many clinical, pathological and
immunological features have been shown. The small intrahepatic bile ducts are a main
target tissue of PBC and hepatocytes are also simultaneously attacked variably.
Serologically, antimitochondrial antibodies (AMA) are characteristically detected in the
patients serum, and the target antigens of AMA are shown to be pyruvate dehydrogenase
E2 complex located in the inner membranes of mitochondria. The term chronic
nonsuppurative destructive cholangitis (CNSDC) more accurately reflects the
pathogenesis of this disease, and mmunopathological features of the bile ducts showing
CNSDC are also detailed. Furthermore, T cell mediated cytotoxicity against biliary
epithelial cells and biliary innate immunity are responsible for the progressive bile duct
damages in PBC. Along with the progressive bile duct damage, chronic cholestasis,
biliary fibrosis and then cirrhosis develop and the patients die of hepatic failure. PBC is a
fatal disease and liver transplantation is only a curable therapy. 

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Primary Biliary Cirrhosis: Pathology and Pathogenesis, pp. 109-144