Laboratory testing revealed elevated liver enzymes, particularly serum alkaline phosphatase and γ-glutamyl-transpeptidase, and positive antinuclear and antimitochondrial antibodies. A liver biopsy confirmed primary biliary cirrhosis.
Primary biliary cirrhosis is a disease of unknown etiology characterized by inflammatory destruction of the small bile ducts and gradual liver cirrhosis. It is much more common in women than men (9:1 ratio).
Regardless of the histologic stage of their cirrhosis or their eventual need for liver transplantation, patients with primary biliary cirrhosis typically are treated with ursodeoxycholic acid, 13 to 15 mg/kg/d. It is given with food 2 to 4 times a day. In a meta-analysis of 7 trials, ursodeoxycholic acid significantly reduced the incidence of liver transplantation. Bile acid sequestrants such as cholestyramine can be used to treat pruritus. This patient was treated with ursodeoxycholic acid and cholestyramine, which relieved her symptoms and improved her liver function test results.
Photo courtesy of Javid Ghandehari, MD. Text for Photo Rounds Friday courtesy of Richard P. Usatine, MD. This case was adapted from: Smith M, Mathia A. Liver disease. In: Usatine R, Smith M, Mayeaux EJ, et al, eds. Color Atlas of Family Medicine. 2nd ed. New York, NY: McGraw-Hill; 2013:377-385.
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