Review Question - QID 106968

This patient's clinical presentation is consistent with primary billiard cirrhosis. First line treatment is ursodeoxycholic acid.

Primary biliary cirrhosis (PBC) is an autoimmune, cholestatic liver disease characterized by the progressive destruction of interlobular bile ducts leading to cirrhosis. Approximately 90% of PBC patients have a positive anti-mitochondrial antibody titer. Other clinical criteria used for the diagnosis of PBC include an alkaline phosphatase greater than 1.5 times the upper limit of normal for at least 24 weeks and the characteristic findings of interlobular bile duct destruction on liver biopsy. Ursodeoxycholic acid functions to protect cholangiocytes and hepatocytes from the cytotoxic effects of bile acids. It is currently the only FDA-approved therapy for PBC.

Heidelbaugh and Bruderly review the causes of cirrhosis and chronic liver failure. Nearly 80 to 90% of the liver parenchyma must be destroyed in order for manifestations of liver failure to present clinically. In PBC, the most important early finding may be a chronic cholestasis on laboratory results. This should prompt abdominal imaging, such as a RUQ ultrasound or contrast cholangiography.

Dyson et al. review novel therapeutic targets in primary biliary cirrhosis. Up to 30% of PBC patients have an inadequate response to ursodeoxycholic acid therapy. Current agents in active clinical trials include budesonide, a corticosteroid that may dampen the inflammatory destruction in PBC; abatacept, which targets the T-cell-mediated immune response in PBC; combination antiretroviral therapy, in response to evidence that PBC may be a result of a retroviral infection; and allogeneic hematopoietic stem cell transplant, which may modulate the immune system response in PBC.

Figure A is pathology slide demonstrating periductal lymphocyte infiltrate and early destructive changes of the bile duct in PBC.

Incorrect Answers:
Answer 1: Endoscopic balloon dilation is commonly employed in the treatment of biliary strictures in patients with primary sclerosing cholangitis.
Answer 2: Liver transplant would not be the initial therapeutic choice in this patient.
Answer 3: As the disease process affects the intrahepatic biliary ducts, a cholecystectomy would not be of significant benefit to a patient with PBC.
Answer 5: This patient is not demonstrating signs of an infectious process such as ascending cholangitis.