Pathology
Histopathologic analyses of liver biopsies of patients with PBC
have resulted in identifying four distinct stages of the disease as
it progresses. The earliest lesion is termed chronic nonsuppurative
destructive cholangitisand is a necrotizing inflammatory process
of the portal tracts. Medium and small bile ducts are infiltrated
with lymphocytes and undergo duct destruction. Mild fibrosis
and sometimes bile stasis can occur. With progression, the inflammatory infiltrate becomes less prominent, but the number of bile
ducts is reduced and there is proliferation of smaller bile ductules.
Increased fibrosis ensues with the expansion of periportal fibrosis to
bridging fibrosis. Finally, cirrhosis, which may be micronodular or
macronodular, develops
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