Primary Biliary Cirrhosis (PBC)

Overview
  • Primary Biliary Cirrhosis (PBC) is a likely autoimmune disease which results in progressive destruction of small to medium-sized bile ducts and ultimately ends in a picture of cirrhosis.
Etiology
  • Several lines of evidence point to an autoimmune etiology. Anti-Mitochondrial Auto-antibodies (AMAs) are present in over 90% of patients and the disease is co-morbid with a number of other autoimmune diseases. Furthermore, injury to bile ducts appears to be the work of auto-reactive lymphocytes.
Morphology
  • The early stages of Primary Biliary Cirrhosis are characterized by a dense, primarily lymphocytic infiltrate into portal tracts and interlobular bile ducts with the occaional presence of non-caseating granulomas. Over time, the bile ducts of inflamed areas are obliterated leading to upstream biliary stasis. Subsequent pathology is due to long-term upstream cholestasis and can eventually end in a picture of secondary biliary cirrhosis.
Clinical Consequences
  • Because of its slow and insidious onset early stages of primary biliary cirrhosis are largely asymptomatic. When symptomology surfaces it is similar to that of general cholestasis characterized by pruritis, jaundice, and xanthomas. Laboratory abnormalities are also similar those of general cholestasis and include conjugated hyperbilirubinemia, elevated serum alkaline phosphatase, and hypercholesterolemia. With progressive disease stigmata of cirrhosis arrive and may lead to hepatic failure.
Incidence
  • Primary Biliary Cirrhosis is primarily presents in middle-aged women.