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The Korean Journal of Pathology 1991;25(4): 318-326.
Primary Biliary Cirrhosis: A Clinicopathologic Analysis of Four Cases.
Mi Kyung Kim, Yong Il Kim, Hyo Suck Lee, Chung Yong Kim
1Department of Pathology, Seoul National University College of Medicine, Seoul, Korea.
2Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.
3Department of Pathology, Dankook University College of Medicine, Seoul, Korea.
ABSTRACT
Primary biliary cirrhosis, a chronic, progressive, and often fatal cholestatic liver disease, is characterized by destruction of intrahepatic bile ducts, portal inflammation and scarring, and the eventual development of cirrhosis and liver failure. We reviewed four cases of primary biliary cirrhosis primarily suspected with peritoneoscopic liver biopsy specimens. All patients were female and their age ranged from 29 to 50 years(median age: 43.8 years). Presenting symptoms were typically pruritus and/or upper abdominal discomfort. On physical examination, interus was presented in 3; hepatomegaly in 3; chronic liver disease stigmata in one. Co-existing autoimmune diseases such as Hashimoto' thyroiditis and Sjoegren' syndrome were found in 2 cases. Biochemical tests of liver function revealed a cholestatic pattern. An antimitochondrial-antibody test was positive in 2 out of 3 patients(75%). Increase levels of serum immunoglobulins, especially of IgM, were evident in 3 cases. Histologic staging(Ludwig et al.) disclosed stage I lesion in one, stage III in two, and stage IV in the remaining one.
Key Words: Primary biliary cirrhosis; Chronic nonsuppurative destructive cholangitis; Liver