Primary Biliary Cirrhosis

The patient had xanthelasma palpebrarum (Figure 1). Xanthomas were noted on the palms and elbows (Figure 2) as well as the nape and left knee. The liver was 2 cm below the costal margin, firm, non-nodular, and nontender, with a span of 10 cm. There was no splenomegaly. Laboratory results showed markedly elevated levels of serum total cholesterol, 941.70 mg/dL; low-density lipoprotein (LDL), 890.73 mg/dL; alkaline phosphatase (ALP), 1301 U/L; alanine aminotransferase (ALT), 138 U/L; albumin, 2.2 g/L; and direct bilirubin, 2.40 mg/dL. The antimitochondrial antibody (AMA) titer was greater than 1:640. These findings suggested primary biliary cirrhosis (PBC). Results of abdominal ultrasonography, CT, and MRI were normal. The patient was treated with ursodiol (15 mg/kg/d in 3 divided doses).BILIARY CIRRHOSIS: AN OVERVIEW Biliary cirrhosis is an autoimmune liver disorder that generally results from injury to or prolonged obstruction of either the intrahepatic or extrahepatic biliary system. It also may be caused by impaired biliary excretion or destruction of small intrahepatic bile ducts and canals of Hering with progressive fibrosis.1,2 PBC is characterized by chronic inflammation as well as fibrous obliteration of the intrahepatic bile ducts; the disease is progressive and can lead to liver damage and ultimately liver failure. In