Most common in individuals of North European descent
• Insidious onset with pruritus, fatigue, jaundice
Often other associated autoimmune disorders
• AMA(+)
Minority of cases are AMA(-)
• Elevation of GGT, alkaline phosphatase
Out of proportion to transaminases
• Ursodeoxycholic acid is treatment of choice
Not cure but delays progression in some patients
Microscopic
• Florid duct lesion with lymphocytic cholangitis and bile duct injury
Granulomatous inflammation variably present
• Biliary epithelial disarray with irregularly sized and pseudostratified nuclei and vacuolated, swollen cytoplasm
• Bile ductular reaction
• Cholate stasis
Copper stain highlights accumulated copper in hepatocytes
• Initially portal-based fibrosis, eventually forms portal-portal bridges
Cirrhosis is biliary type with irregular nodules (so-called jigsaw puzzle pattern)
• 4 histologic stages ranging from portal involvement to cirrhosis
Gross Specimen This photograph of the cut surface of a liver explanted for primary biliary cholangitis (PBC) emphasizes the green discoloration of the cirrhotic nodules, indicative of chronic cholestasis.
Florid Duct Lesion This example of a florid duct lesion shows lymphocytic cholangitis in the interlobular bile duct along with a nodular portal lymphoplasmacytic infiltrate and a granulomatous inflammation.
Portal Tract Changes This biopsy from a patient with PBC shows nodular lymphoplasmacytic inflammation and lymphocytic cholangitis, even at low power. Fibrous expansion of the portal tract is also present. Similar histologic changes are also characteristic of AMA PBC (autoimmune cholangitis).
Copper Stain The copper stain highlights changes of chronic cholestasis consisting of periportal orange-red granules in the hepatocytes in PBC.
TERMINOLOGY
Abbreviations
• Primary biliary cirrhosis/cholangitis (PBC)
Cholangitis is recently preferred term
Definitions
• Chronic cholestatic disease in which intrahepatic bile ducts are progressively destroyed by nonsuppurative inflammation
ETIOLOGY/PATHOGENESIS
Unknown
• Probable autoimmune etiology
CLINICAL ISSUES
Epidemiology
• Incidence
Most common in individuals of North European descent
along with a nodular portal lymphoplasmacytic infiltrate and a granulomatous inflammation.
in the hepatocytes in PBC.
