Autoimmune liver diseases involve a misguided immune system response
that results in an attack on normal healthy tissues of the liver. Often
the cause is unknown but believed to involve a combination of genetic
and other risk factors. The autoimmune response causes chronic
inflammation that can lead to destruction and loss of function of the
liver. There are three important autoimmune diseases of the liver, all
of which can progress to cirrhosis.
Autoimmune hepatitis involves chronic inflammation and damage of liver
cells. Diagnosis is based on symptoms, blood tests, and a liver biopsy.
Treatment typically is lifelong therapy with medications that suppress
the immune system (prednisone, azathioprine).
Primary Biliary Cirrhosis
Primary biliary cirrhosis involves chronic inflammation and damage of
the bile ducts inside the liver. The bile ducts may be destroyed,
causing bile to build up in the liver. If primary biliary cirrhosis is
suspected, blood tests and, possibly, a liver biopsy are performed to
make the diagnosis. Medication (ursodiol) can help slow the disease, but
some people may develop end-stage liver disease. Liver transplantation
may be an option.
Primary Sclerosing Cholangitis (PSC)
Primary sclerosing cholangitis (PSC) involves chronic inflammation and
damage of the bile ducts both inside and outside the liver, leading to a
buildup of bile in the liver. In addition to cirrhosis, PSC can lead to
bile duct cancer (cholangiosarcoma).
If PSC is suspected, the diagnostic evaluation may include blood tests,
imaging studies, a liver biopsy, and endoscopic retrograde
cholangiopancreatography (ERCP). Currently, there are no specific
treatments for PSC. Medications are used to treat symptoms and
complications, and ERCP procedures can be used to help unblock bile
ducts. Liver transplantation may be an option for advanced PSC.
For more information, call 1.866.CALL.MLH.