Secondary Biliary Cirrhosis

Overview
  • Secondary Biliary Cirrhosis refers to a unique pattern of cirrhosis which develops in the liver secondary to long-term extra-hepatic cholestasis. In most cases, extra-hepatic cholestasis is due to cholelithiasis or neoplastic infiltration of the extra-hepatic biliary tree.
Pathogenesis
  • Long-term extra-hepatic cholestasis results in backup of bile into the intra-hepatic biliary tree leading to hepatic pathology. The progressive morphological features leading to hepatic fibrosis and ultimately cirrhosis are the same as those generically discussed in cholestasis (see page).
Morphology
  • The end-stage liver due to secondary biliary cirrhosis displays nodules of hepatocytes divided by thick fibrotic septae. Within these fibrotic septae, proliferating bile ductules filled with bile may exist. Hepatocytes within nodules display "Feathery Degeneration" referring to their possession of bile droplets which can no longer be excreted.
Clinical Consequences
  • Patients will display the general clinical features of cholestasis such as pruritis, xanthomas, jaundice, steatorrhea, and deficiencies of the fat soluble vitamins A, D, E, and K. Over time patients will display stigmata and complications of cirrhosis.