Description

Ludwig and Batts identified different histologic stages in liver biopsies from patients with primary sclerosing cholangitis. The scheme is similar to the one used for primary biliary cirrhosis (above).


 

Histologic Features

Stage

lymphocytic portal hepatitis; periportal change absent or minimal

1

periportal hepatitis with fibrosis; early loss of bile ducts; ductular proliferation

2

bridging fibrosis with formation of fibrous septa; moderate to marked ductopenia; ductular proliferation

3

cirrhosis with regenerative nodules which may be typical or "garland shaped"; moderate to marked ductopenia; ductular proliferation; cholestasis with feathery degeneration

4

 

where:

• Fibrous obliterative cholangitis is uncommon in Stages 1 and 2 but is common in wedge biopsies for Stages 3 and 4.

• Mallory's hyaline may be common in Stage 4.

 

Limitations:

• The pathologic changes may not be evenly distributed within the liver. Therefore a stage determined on a limited sampling such as needle biopsy may differ from that seen with a more extensive sampling.

 


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