Autoimmune Liver Disease
What is Autoimmune Liver Disease?
Autoimmune liver diseases are chronic, inflammatory diseases that usually progress slowly. The three main categories of autoimmune liver disease are autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC).
Primary biliary cholangitis (PBC) is also a liver disease with no known cause but is believed to be related to other autoimmune disorders. Primary biliary cholangitis begins with inflammation of the small bile ducts. The inflammation blocks the flow of bile out of the liver and liver cells, causing inflammation. As inflammation spreads, scar tissue (fibrosis) develops throughout the liver, replacing more and more liver tissue with fibrosis (scarring), leading to cirrhosis in some cases.
Primary Sclerosing Cholangitis (PSC) is a disease of the large bile ducts in which they become inflamed and heavily scarred. Its cause is unknown and at this time there is no treatment known to stop the disease from progressing.
What are the Symptoms?
It is often associated with other autoimmune disorders, such as Sjogren’s syndrome (dry eyes, dry mouth), Raynaud’s phenomenon (impaired circulation in hands and feet in response to cold), hyperthyroidism (Graves’ disease or Hashimoto’s thyroiditis) and rheumatoid arthritis.
Most people with AIH do not experience symptoms until their disease is advanced, but early symptoms may include fatigue. AIH sometimes begins with a “flare” with jaundice, fatigue, itching, fever, and right upper quadrant discomfort
How is it Diagnosed and Treated?
Untreated AIH can lead to progressive scarring of the liver (including cirrhosis).
When diagnosed and treated early, AIH often can be controlled with drugs that suppress the immune system, including steroids and azathioprine.
A liver transplant may be an option if autoimmune hepatitis progresses to advanced liver disease.
Diagnosis of AIH maybe made by blood tests (Anti-nuclear Antibody, Smooth Muscle Antibody), but often a liver biopsy is required.