Autoimmune Liver Disease
Autoimmune Disorders
- Immune system abnormalities: Your immune system protects your body from germs and toxins. But that system can attack certain parts of your body (autoimmune), including your liver; this is called autoimmune disease. General examples of autoimmune diseases are rheumatoid arthritis and inflammatory bowel disease. Examples of autoimmune liver diseases include autoimmune hepatitis, primary biliary cholangitis, primary sclerosing cholangitis, and others.
- There are two types of autoimmune liver diseases, each with different types of autoantibodies:
- Type 1 (anti-nuclear (ANA) and/or anti-smooth muscle (SMA) antibodies) make up two out of three cases of autoimmune hepatitis (AIH) and most autoimmune sclerosing cholangitis (ASC) cases.
- Type 2 (liver kidney microsomal (LKM) antibodies) is less common, but more likely to affect younger children and can cause acute liver failure (ALF). Type 2 is rarely associated with ASC cases.
Primary Sclerosing Cholangitis (PSC)
- Primary sclerosing (skluh-ROHS-ing) cholangitis (koh-lan-JIE-tis) (PSC) is a rare chronic liver disease of the bile ducts. Bile ducts, located inside and outside the liver, carry bile from the liver to the small intestine for digestion. With PSC, the liver’s bile ducts become inflamed and scarred, causing them to become narrowed or blocked, seriously damaging the liver. In 10 to 15 years, this can lead to liver failure. Many people with PSC also have inflammatory bowel disease (IBD), including ulcerative colitis or Crohn's disease.
- The causes of PSC are not clear. Indications are for an immune system reaction to an infection or toxin in people with a genetic predisposition to the disease.
- PSC is rare. Incidence rates for PSC range from 1.3 per 100,000 to 1 in 10,000 people per year worldwide.
- Prevalence rates for PSC are approximately 1 to 16 per 100,000 people in the U.S.
- PSC is usually diagnosed between the ages of 30 and 40 years. It is more common in men, who have a two-fold risk compared to women. Family history of the disease is also a risk factor.
- PSC is more common in North America and Northern Europe than in Asia.
- PSC has no "type 1" or "type 2" distinctions; PSC diagnosis uses a system with stages 1-4, based on the progression of the disease. Stage 1 is minimal fibrosis (scarring) around the portal areas of the liver, eventually progressing to more advanced fibrosis and cirrhosis (stage 4) in later stages (stage 4) These stages are diagnosed using a liver biopsy.
- Stages of PSC:
- Stage 1 – A small amount of fibrosis limited mostly to portal regions of the liver.
- Stage 2 – Fibrosis outside portal areas. The strands of fibrosis not connected.
- Stage 3 – Fibrosis areas connecting to each other.
- Stage 4 – Widespread honeycomb-like scarring, cirrhosis.
- IMPORTANT: Because of the similarity of the abbreviations/acronyms, it is easy to confuse PSC with PBC. Remember: PSC = primary sclerosing cholangitis; PBC = primary biliary cholangitis.
Primary Biliary Cholangitis (PBC)
- IMPORTANT: The full name of the disease “PBC” has been changed from “primary biliary cirrhosis” to “primary biliary cholangitis.”
- Primary biliary cholangitis (PBC; formerly “primary biliary cirrhosis”) is a chronic disease of the small bile ducts in the liver. The ducts become injured and are eventually destroyed. When there are no bile ducts, bile backs up, causing liver damage. It can be life-threatening if left untreated.
- Possible causes of PBC include autoimmunity, infection, or genetic predisposition.
- IMPORTANT: Because of the similarity of the abbreviations/acronyms, it is easy to confuse PSC with PBC. Remember: PSC = primary sclerosing cholangitis; PBC = primary biliary cholangitis.
- Primary biliary cholangitis (PBC) is rare. The reported prevalence varies from 19 to 402 cases per million people.
- PBC mostly afflicts women (90%-95%). Women are affected 10 times more than men, but recent studies indicate that the ratio may be closer to 4:1 – 6:1.
- Most people are diagnosed with PBC between ages 30 and 65 years, often in their 40s or 50s. The disease has also been reported in women aged 15 years and 93 years.
- One 2014 study found a PBC prevalence of 58 in every 100,000 U.S. women, and approximately 15 out of every 100,000 U.S. men.
- There is no cure for PBC, but treatment can help slow the disease’s progression and prevent complications.
- Cholestatic pruritus – severe, intense itching – is a key symptom of PBC, experienced by up to 81% of PBC patients.
Autoimmune Hepatitis (AIH)
- Autoimmune hepatitis is when your immune system attacks your liver, causing inflammation, swelling, and liver damage. It is a chronic disease that can lead to cirrhosis and liver failure.
- The causes are unknown, but AIH is more likely in people with other autoimmune conditions. Genetic and environmental factors and some medications can trigger autoimmune hepatitis.
- AIH affects women more than men.
- If AIH is diagnosed and treated early, it can be controlled with medicines that suppress the immune system. The disease can go into remission.
- People with other autoimmune conditions have a 25-50% chance of developing another autoimmune disorder and are at higher risk for developing AIH. (data 2016, 2019)
- There are two types of AIH: type 1 and type 2. Type 1 AIH, also called the “classic type,” is typically diagnosed in adulthood; type 2 is diagnosed during childhood.
- Life expectancy with autoimmune hepatitis: Without treatment, 50% within 5 years; with treatment, 90% in 10 years and 70% in 20. About 15% of people being treated eventually develop cirrhosis, usually after 10 to 20 years.
- AIH incidence has been estimated at 1-2 per 100,000 people. (data 2016, 2019)
- AIH prevalence has been estimated at 24 per 100,000. (data 2016, 2019)
- Studies suggest that the incidence of AIH is increasing for unknown reasons.
- An estimate of the prevalence of AIH in the US was 31.2/100,000. (2014-2019)
- Result of a global pooled prevalence study of AIH was 15.65 cases per 100,000. (2023)
- The incidence of AIH was greater in North America and Oceania (compared with Asia), among females, adults (vs. children).
Autoimmune Sclerosing Cholangitis (ASC)
- Autoimmune sclerosing cholangitis (ASC), also known as “overlap syndrome (OS),” refers to an autoimmune condition with characteristics of both autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) or primary biliary cholangitis (PBC).
- The prevalence of AIH-PSC overlap (ASC) ranges from 1.7%–10% in adult patients with AIH.
- The prevalence of ASC in children is much higher ranging from 20%–to 49%.
- Autoimmune sclerosing cholangitis (ASC) in children is frequently associated with inflammatory bowel syndrome (IBS).
Medically reviewed on April 2025.
Last updated on December 11th, 2025 at 11:29 am