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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.
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.
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.
Autoimmune hepatitis is diagnosed by:
Primary biliary cirrhosis is diagnosed by:
Primary sclerosing cholangitis is diagnosed by:
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.
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.
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.
Learn more about autoimmune hepatitis, primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) at the American Liver Foundation:
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