Overview

Autoimmune hepatitis is a liver disease that happens when the body's immune system attacks the liver. This can cause swelling, irritation and damage to the liver. The exact cause of autoimmune hepatitis is unclear, but genetic and environmental factors appear to interact over time to trigger the disease.

Untreated autoimmune hepatitis can lead to scarring of the liver, called cirrhosis. It can also eventually lead to liver failure. When diagnosed and treated early, however, autoimmune hepatitis often can be controlled with medicines that suppress the immune system.

A liver transplant may be an option when autoimmune hepatitis doesn't respond to medicines or liver disease becomes advanced.

Symptoms

Symptoms of autoimmune hepatitis vary from person to person and may come on suddenly. Some people have few, if any, recognized problems in the early stages of the disease, whereas others experience symptoms that may include:

  • Fatigue.
  • Belly discomfort.
  • Yellowing of the skin and whites of the eyes, called jaundice. Depending on skin color, this change may be harder or easier to see.
  • An enlarged liver.
  • Irregular blood vessels on the skin, called spider angiomas.
  • Skin rash.
  • Joint pain.
  • Loss of menstrual periods.

When to see a doctor

Make an appointment with a healthcare professional if you have any symptoms that worry you.

Causes

Autoimmune hepatitis occurs when the body's immune system, which usually attacks viruses, bacteria and other causes of disease, instead targets the liver. This attack on the liver can lead to long-lasting inflammation and serious damage to liver cells. Just why the body turns against itself is unclear, but researchers think autoimmune hepatitis could be caused by the interaction of genes controlling immune system function and exposure to viruses or medicines.

Types of autoimmune hepatitis

Experts have identified two main forms of autoimmune hepatitis.

  • Type 1 autoimmune hepatitis. This is the most common type of the disease. It can occur at any age. About half the people with type 1 autoimmune hepatitis have other autoimmune disorders, such as celiac disease, rheumatoid arthritis or ulcerative colitis.
  • Type 2 autoimmune hepatitis. Although adults can develop type 2 autoimmune hepatitis, it's most common in children and young people. Other autoimmune diseases may accompany this type of autoimmune hepatitis.

Risk factors

Factors that may increase your risk of autoimmune hepatitis include:

  • Being female. Although both males and females can develop autoimmune hepatitis, the disease is most common in females.
  • Genetics. Evidence suggests that a predisposition to autoimmune hepatitis may run in families.
  • Having an autoimmune disease. People who already have an autoimmune disease, such as celiac disease, rheumatoid arthritis or hyperthyroidism (Graves' disease or Hashimoto thyroiditis), may be more likely to develop autoimmune hepatitis.

Complications

Autoimmune hepatitis that goes untreated can cause permanent scarring of the liver tissue, known as cirrhosis. Complications of cirrhosis include:

  • Enlarged veins in the esophagus, called esophageal varices. The portal vein carries blood from the intestine to the liver. When circulation through the portal vein is blocked, blood may back up into other blood vessels, mainly those in the stomach and esophagus.

    These blood vessels have thin walls. And because they become filled with more blood than they're meant to carry, they're likely to bleed. Massive bleeding in the esophagus or stomach from these blood vessels is a life-threatening emergency that needs immediate medical care.

  • Fluid in the abdomen, called ascites (uh-SY-teez). Liver disease can cause large amounts of fluid to build up in the belly. Ascites can be uncomfortable and may interfere with breathing. It's usually a sign of advanced cirrhosis.
  • Liver failure. Liver failure happens when extensive damage to liver cells makes it not possible for the liver to function well. At this point, a liver transplant is needed.
  • Liver cancer. People with cirrhosis have an increased risk of liver cancer.
By Mayo Clinic Staff