A liver biopsy may be relatively nonspecific and show only inflammation with interlobular and septal fibrosis. A biopsy is most useful as an indicator of the disease stage. Many patients may be asymptomatic, but still have advanced disease. Symptoms of itching, fatigue, jaundice, and weight loss are often indicators of advanced as well. The natural history of primary sclerosing cholangitis unfortunately involves a slow, silent progression to cirrhosis, portal hypertension, and eventual liver failure.
Liver biopsies can follow the progress through four stages.
Stage 1 incorporates inflammation, scarring, and enlargement of portal triads. As the inflammation becomes more widespread and infiltrates the parenchyma, it is classified as stage 2. Treatments for primary sclerosing cholangitis are of variable effectivity. None have clearly been shown to prolong life, though some do improve LFT abnormalities. It is a replacement bile acid considered to be less hepatotoxic than the biliary secretions that accumulate in cholestatic disease.