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Daniel S. Pratt, MD, director of the Autoimmune and Cholestatic Liver Clinic, answers common questions about the cause of and treatments for autoimmune hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis.

What are autoimmune hepatitis, primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC)?

Autoimmune disease of any form means an individual’s own immune system interprets normal body tissue to be foreign. The result is that the immune system attacks the tissue it views as hostile. In autoimmune hepatitis the immune system attacks the liver cells called hepatocytes. In PBC and PSC the immune system attacks the bile duct cells, called cholangiocytes, which help digest food.

What causes these conditions?

The exact causes of these conditions remain unknown. However, it is believed that some patients have genes that increase their risk of developing an autoimmune disease, but they only develop an autoimmune disease if they are exposed to the right environmental trigger. Those triggers are unknown, but theories include common chemicals and medications as well as bacterial and viral infections.

How are these conditions diagnosed?

Autoimmune hepatitis is diagnosed by:

  • The presence of the typical pattern of liver test abnormalities (elevated alanine aminotransferase (ALT) and aspartate aminotransferase (AST)
  • Elevated autoimmune markers in the blood (antinuclear antibody (ANA), smooth muscle antibody (ASMA), and an elevated immune protein called IgG)
  • A liver biopsy showing an immune attack on the liver cells

Primary biliary cirrhosis is diagnosed by:

  • The presence of the typical pattern of liver test abnormalities (an elevated serum alkaline phosphatase)
  • The presence of antimitochondrial antibodies (AMA) in the blood
  • A liver biopsy that shows the characteristic immune attack on the smallest bile ducts in the liver

Primary sclerosing cholangitis is diagnosed by:

  • The presence of the typical pattern of liver test abnormalities (an elevated serum alkaline phosphatase)
  • A cholangiogram (a picture of the bile ducts) that shows narrowings, known as strictures. The cholangiogram can be obtained either with an MRI or endoscopically with an endoscopic retrograde cholangiography (ERCP)
Do these conditions run in families?

Autoimmune conditions in general can run in families, but it is rare that any of these autoimmune liver diseases occur in more than one family member.

How are these conditions treated?

Autoimmune liver disease is treated with a course of medication used to suppress the immune system. This is usually the medication called prednisone, followed by azathioprine. PBC is treated with a medication called ursodeoxycholic acid and perhaps other medications depending on your circumstances. There is no proven medical therapy for PSC.

Can these conditions cause cirrhosis of the liver?

Yes, cirrhosis, or scarring of the liver that results in poor function, can develop with any of these conditions. However, if caught early enough and treated aggressively, autoimmune hepatitis should not progress to cirrhosis. The majority of patients with PBC also respond to current medical therapies and are less likely to develop cirrhosis. Because there are currently no treatments for PSC that are proven to be effective, cirrhosis eventually develops in the majority of patients.