The exact causes of autoimmune liver diseases are not fully understood. It is believed that a combination of genetic factors and environmental triggers may play a role in why the immune system mistakenly attacks the liver. 

There are three main types: Autoimmune Hepatitis (AIH), Primary Biliary Cholangitis (PBC), and Primary Sclerosing Cholangitis (PSC). In PBC and PSC, the immune system primarily targets the bile ducts, which carry bile from the liver to the intestines. In AIH, it attacks the liver cells themselves (called hepatocytes).

To diagnose autoimmune liver diseases, doctors use a combination liver function tests, special blood tests, imaging, and sometimes a liver biopsy.

Blood tests look for autoantibodies, these are proteins made by the immune system that mistakenly target the body’s own tissues.

For example:

Autoimmune Hepatitis (AIH) is often suspected when certain autoantibodies are found in the blood, but a liver biopsy (taking a small sample of liver tissue) is usually needed to confirm the diagnosis.

Primary Biliary Cholangitis (PBC) is mainly diagnosed through blood tests that detect specific autoantibodies.

Primary Sclerosing Cholangitis (PSC) is diagnosed using a special imaging test called Magnetic Resonance Cholangiopancreatography (MRCP), which creates detailed pictures of the bile ducts.

Treatment depends on the type of autoimmune liver disease but usually involves medications to control the immune system. While there is no cure, treatment aims to manage the disease, reduce inflammation, and prevent further liver damage. Close monitoring is important to track how well the treatment is working and to minimize any side effects from long-term therapy.

There is ongoing research to better understand these diseases and develop new therapies that may improve treatment options in the future. A liver specialist can help guide you through your diagnosis, treatment plan, and connect you with the latest advances in care.

Unfortunately, there is currently no way to prevent autoimmune liver diseases. Therefore, the best way remains to detect these diseases early through regular testing, before permanent scarring of the liver (cirrhosis) has occurred.