Table 1.
Histopathological features | PSC/BS | Chronic rejection | Primary biliary cirrhosis |
Distribution, severity and composition of portal inflammation | Usually patchy to diffuse; mild neutrophilic, eosinophilic, or occasionally mononuclear predominant | Patchy; usually minimal or mild lymphoplasmacytic | Noticeably patchy and variable intensity; predominantly mononuclear; nodular aggregates and granulomas |
Presence and type of interface activity | Prominent and defining feature: ductular type with portal and periportal edema | Minimal to absent | Important feature later in disease development: ductular and necroinflammatory-type with copper deposition |
Bile duct inflammation and damage | Periductal lamellar edema "fibrous cholangitis"; acute cholangitis; multiple intra-portal ductal profiles | Focal ongoing lymphocytic bile duct damage; inflammation wanes with duct loss | Granulomatous or focally severe lymphocytic cholangitis is diagnostic in proper setting |
Biliary epithelial senescence changes and small bile duct loss | Small bile duct loss associated with ductular reaction | Senescence/atrophy/atypia involve a majority of remaining ducts | Small bile duct loss associated with ductular reaction |
Perivenular mononuclear inflammation and/or hepatocyte dropout | Absent | Usually present but variable | Variable but generally mild; if present, involves a minority of perivenular regions |
Lobular findings and necroinflammatory activity | Disarray unusual; neutrophil clusters; ± cholestasis | Variable; if present, concentrated in perivenular regions | Mild disarray; parenchymal granulomas; periportal copper deposition and cholestasis are late features |
Pattern of fibrosis during progression towards cirrhosis | Biliary pattern | Uncommon; if present usually a venocentric pattern; may evolve to biliary pattern | Biliary pattern |
PSC: Primary sclerosing cholangitis; BS: Biliary strictures.