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Date published: July 7, 2023

Autoimmune and

Cholestatic Liver Disease


Autoimmune liver diseases arise when the body’s immune system attacks the liver which causes inflammation. Without treatment, the liver inflammation could eventually cause cirrhosis of the liver, which may then lead to liver cancer and liver failure.

There are three main types of autoimmune liver diseases:

  1. Autoimmune hepatitis. Occurs when your body’s immune system attacks your liver cells. Often asymptomatic with no symptoms before liver failure, it affects both children and adults and can lead to cirrhosis and acute liver failure.

  2. Primary biliary cirrhosis (PBC). This condition is the result of the body’s own immune cells attacking and destroying the biliary ducts within the liver. Symptoms may include itching, dry eyes, dry mouth, fatigue, bone disease or high cholesterol or it may be asymptomatic (no symptoms). Without treatment, cirrhosis and liver failure could occur.

  3. Primary sclerosing cholangitis (PSC). Known to cause inflammation and scarring of the bile ducts which can prevent bile from passing through. Consistent with other autoimmune liver diseases, symptoms could be nonexistent or linked to symptoms like itching or jaundice. A frequent association is found with IBS (inflammatory bowel disease). Complications of PSC involve cancers of the liver, bile duct and gallbladder in addition to biliary infections and cirrhosis.

Primary biliary cirrhosis and primary sclerosing cholangitis are also the two most common chronic cholestatic liver diseases affecting adults. This term comes from cholestasis, a liver disease which occurs when the flow of bile from the liver is reduced or blocked. Causes of this are divided into two groups: within the liver and outside the liver. Regardless of the cause, if left untreated cholestasis will lead to fibrosis, biliary cirrhosis and finally, end-stage liver disease.

Diagnosis & Treatment

Patients with these conditions are often misdiagnosed or worse, go undiagnosed due to the complex presentations of these diseases. In some cases, there is an overlap between these conditions, adding to the complicated nature of achieving a correct and thorough diagnosis.

Methodist Transplant Specialists offer an expert-level, multidisciplinary approach to diagnosing and treating these diseases. Blood testing is most often the first diagnostic step due to the fact that patients do not often present with symptoms until their disease has progressed to cirrhosis or liver failure.

In addition, our team may have you undergo diagnostic imaging of your liver and if necessary a liver biopsy.

The treatment for these autoimmune liver diseases are varied and depend on your exact diagnosis:

  • Autoimmune liver disease treatment is aimed at suppressing an overactive immune system

  • PBC treatment will focus on improving the flow of bile

  • Currently, their is active clinical research on therapies for PSC

  • Advanced autoimmune liver disease may require a liver transplant

Due to the fact that patients with autoimmune disease often have increased risk for autoimmune liver disease, our team at Methodist Transplant Specialists will coordinate all medical and clinical histories, diagnostic results and any other relevant information to ensure you receive a proper diagnosis.