Korean J Hepatol.  2011 Sep;17(3):173-179. 10.3350/kjhep.2011.17.3.173.

The diagnosis and treatment of primary biliary cirrhosis

Affiliations
  • 1Department of Internal Medicine, Inje University Ilsan Paik Hospital, Inje University College of Medicine, Goyang, Korea.
  • 2Department of Internal Medicine, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea. jsh@snuhbh.org

Abstract

Primary biliary cirrhosis (PBC) is a slowly progressive cholestatic liver disease of autoimmune etiology. The initial presentation of PBC is various from asymptomatic, abnormal liver biochemical tests to overt cirrhosis. The diagnosis of PBC is based on cholestatic biochemical liver tests, presence of antimitochondrial antibody and histologic findings of nonsuppurative destructive cholangitis. Although the diagnosis is straightforward, it could be underdiagnosed because of its asymptomatic presentation, or underrecognition of the disease. UDCA in a dose of 13-15 mg/kg is the widely approved therapy which can improve the prognosis of patients with PBC. However, one-third of patients does not respond to UDCA therapy and may require liver transplantation. Every effort to diagnose PBC in earlier stage and to develop new therapeutic drugs and clinical trials should be made.

Keyword

Primary biliary cirrhosis; Antimitochonrial antibody; Ursodeoxycholic acid

MeSH Terms

Autoantibodies/blood
Autoimmunity/immunology
Cholagogues and Choleretics/therapeutic use
Humans
Liver Cirrhosis, Biliary/*diagnosis/pathology/*therapy
Liver Transplantation
Ursodeoxycholic Acid/therapeutic use
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