COMMENTARY

Primary Sclerosing Cholangitis -- Approach to Diagnosis

Ian L. Steele, MD; Cynthia Levy, MD; Keith D. Lindor, MD

Disclosures

April 25, 2007

Histopathology

Because histologic findings in PSC tend to be nonspecific, liver biopsy is used primarily for staging the disease.[35,36] The staging is based on the degree of inflammation, ductal proliferation, and ductopenia. In general, early PSC is marked by inflammation and later, as the disease progresses, fibrosis predominates. Eventually the bile duct can be replaced by a solid fibrous cord.[2,36,37] However, it is sometimes difficult to stage PSC histologically because various manifestations of the disease can occur within the same liver. Further complicating this issue, the classic description of periductal "onion skin" fibrosis (Figure 3) is present in less than 40% of biopsy specimens and is not pathognomonic for PSC.[38]

Figure 3.

Liver biopsy from a patient with stage II PSC showing concentric periductal fibrosis ("onion skin") involving interlobular bile duct; hematoxylin-eosin, X40.

The PSC staging system is similar to that used for primary biliary cirrhosis[38]:

Stage I (portal stage) -- focal inflammation limited to portal triad, with or without bile duct abnormalities, without fibrosis;

Stage II (periportal stage) -- enlargement of portal tracts, periportal fibrosis with or without periportal inflammation;

Stage III (septal stage) -- extension of septal or bridging fibrosis with bridging necrosis; and

Stage IV -- biliary cirrhosis.

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