Autoimmune liver diseases occur when the body’s immune system attacks the liver, causing inflammation. If left untreated, the liver inflammation may eventually cause cirrhosis of the liver, which may lead to liver cancer and liver failure.  

Overview and Symptoms

Although a number of autoimmune conditions may involve the liver, the three most common autoimmune liver diseases are autoimmune hepatitis, primary biliary cholangitis and primary sclerosing cholangitis. These conditions may occur individually or as part of “overlap” syndromes.

  • Autoimmune hepatitis (AIH) can affect both children and adults and can lead to cirrhosis and acute liver failure. It is often asymptomatic, with no symptoms prior to liver failure. It may also be associated with nonspecific symptoms including fatigue, nausea, abdominal pains, or joint pains.
  • Primary biliary cholangitis (PBC) occurs when the biliary ducts within the liver are destroyed by the body’s own immune cells, impairing the liver’s ability to excrete bile. It affects women more often the men. It may be asymptomatic or may be associated with symptoms such as fatigue, itching, dry mouth, dry eyes, high cholesterol or bone disease. It may occur in association with other autoimmune conditions. If left untreated, PBC can lead to cirrhosis and liver failure.
  • Primary sclerosing cholangitis (PSC) is characterized by inflammation and scarring of the bile ducts, which can prevent bile from passing through. Like other autoimmune liver diseases, it may be asymptomatic or may be associated with symptoms such as itching or jaundice. PSC can affect both children and adults and is frequently encountered in association with inflammatory bowel disease. Complications of PSC include liver, gallbladder and bile duct cancers, as well as biliary infections and cirrhosis.

Treatment at BIDMC

Blood testing is often the first step to diagnosing autoimmune liver diseases because many patients do not show symptoms until the disease has progressed to cirrhosis or liver failure. Your doctor may request diagnostic imaging of your liver and may also perform a liver biopsy.

Individuals with another autoimmune disease may be at higher risk for autoimmune liver diseases. Physicians within the Autoimmune and Cholestatic Liver Disease Clinic at BIDMC incorporate all of your clinical history and diagnostic studies to make the most appropriate diagnosis.

Treatments for autoimmune liver disease vary depending on the specific diagnosis.

  • For AIH, treatment will focus on suppressing an overactive immune system
  • For PBC and other cholestatic liver diseases, treatment is aimed at improving bile flow.
  • Therapy for PSC is an area of active research.
  • For advanced autoimmune liver diseases, liver transplantation may be required.

Learn More

The Liver Center at BIDMC brings together an exceptional team of multidisciplinary providers to offer personalized care and the most advanced treatment options to patients with a variety of liver conditions.

Liver Center