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KMID : 1103920060120030364
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Korean Journal of Hepatology
2006 Volume.12 No. 3 p.364 ~ p.372
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Primary Biliary Cirrhosis
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Chon Chae-Yoon
Park Jun-Yong
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Abstract
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Primary biliary cirrhosis (PBC) is a chronic cholestatic autoimmune liver disease that predominantly affects
middle-aged women. It is characterized by slowly progressive destruction of the small intrahepatic bile ducts
together with portal inflammation, and this initially leads to fibrosis and later to cirrhosis. It is currently
accepted that the pathogenesis of PBC is multifactorial with genetic and environmental factors interplaying to
determine the disease onset and progression. In addition to antimitochondrial antibody (AMA), which is the
hallmark of PBC and is detected in at least 90% of the patients, other autoantibodies (antinuclear antibody,
anti-smooth muscle antibody and rheumatoid factor, etc.) may also be found in the patients. There is no
correlation between the titer of AMAs and the disease severity. Most patients are diagnosed either during the
asymptomatic phase of PBC or after presenting with non-specific symptoms. Pruritus and fatigue are the most
common symptoms of PBC. The prognosis of PBC has improved significantly during the last few decades.
Patients are now diagnosed earlier in its clinical course, they are more likely to be asymptomatic at diagnosis
and they are more likely to receive medical treatment. A wide variety of drugs have been assessed for the
treatment of this condition: such immunosuppressive agents as corticosteroids, cyclosporine and azathioprine
have a weak effect on the disease’s natural history. Ursodeoxycholic acid (UDCA) is the only currently
approved medical treatment. For PBC patients with end-stage liver disease or an unacceptable quality of life,
liver transplantation is the only accepted therapeutic option. Early diagnosis and treatment of PBC are
important because effective treatment with UDCA has been shown to delay disease progression and improve
rate survival in the early stage.
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KEYWORD
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Primary biliary cirrhosis, Prevalence, Diagnosis, Therapeutics
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