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Autoimmune Hepatitis

Overview

Autoimmune hepatitis is liver inflammation that occurs when your body's immune system turns against liver cells. The exact cause of autoimmune hepatitis is unclear, but genetic and enviromental factors appear to interact over time in triggering the disease.

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

A liver transplant may be an option when autoimmune hepatitis doesn't respond to drug treatments or in cases of advanced liver disease.

Symptoms

Signs and 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 signs and symptoms that may include:

  • Fatigue
  • Abdominal discomfort
  • Yellowing of the skin and whites of the eyes (jaundice)
  • An enlarged liver
  • Abnormal blood vessels on the skin (spider angiomas)
  • Skin rashes
  • Joint pains
  • Loss of menstrual periods

Causes

Autoimmune hepatitis occurs when the body's immune system, which ordinarily attacks viruses, bacteria and other pathogens, instead targets the liver. This attack on your liver can lead to chronic 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 particular viruses or drugs.

Types of autoimmune hepatitis

Doctors 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 more common in females.
  • A history of certain infections. Autoimmune hepatitis may develop after you're infected with the measles, herpes simplex or Epstein-Barr virus. The disease is also linked to hepatitis A, B or C infection.
  • Heredity. 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's thyroiditis), may be more likely to develop autoimmune hepatitis.

Complications

Esophageal varices

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Autoimmune hepatitis that goes untreated can cause permanent scarring of the liver tissue (cirrhosis). Complications of cirrhosis include:

  • Enlarged veins in your esophagus (esophageal varices). When circulation through the portal vein is blocked, blood may back up into other blood vessels — mainly those in your stomach and esophagus. The blood vessels are thin walled, and because they're 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 requires immediate medical care.
  • Fluid in your abdomen (ascites). Liver disease can cause large amounts of fluid to accumulate in your abdomen. Ascites can be uncomfortable and may interfere with breathing and is usually a sign of advanced cirrhosis.
  • Liver failure. This occurs when extensive damage to liver cells makes it impossible for your liver to function adequately. At this point, a liver transplant is needed.
  • Liver cancer. People with cirrhosis have an increased risk of liver cancer.

Who is at risk for autoimmune hepatitis?

Autoimmune hepatitis affects one in every 100,000 people. The disease is more common in women than in men, and women are typically diagnosed in their 40s or 50s. Girls between two and 14 years old may also get the disease.

Those who may suffer from other autoimmune conditions, such as diabetes, rheumatoid arthritis, thyroid disease and celiac disease, are also at risk to develop autoimmune hepatitis (just as those with autoimmune hepatitis are also at higher risk to develop other autoimmune diseases).

A person who has a family history of autoimmune hepatitis also has a higher risk of developing the disease.

Remission

After receiving treatment for AIH, some people may go into remission. Remission means that a person:

  • no longer has AIH symptoms
  • has a liver with improved function
  • is no longer experiencing liver damage
  • Once a person is in remission from AIH, a doctor may reduce their medications or stop treating them.
  • However, being in remission does not mean that a person is cured of AIH. Without certain treatments, the risk of AIH returning is more than 80%. This is why it is important that a doctor carefully monitors a person with AIH.

Liver transplant

If a person’s AIH progresses and causes liver failure or severe liver scarring, they may require a liver transplant. The Genetic and Rare Diseases Information Center (GARD) states that around 10⁠–20% of people with AIH will need a liver transplant.

A liver transplant involves surgeons removing a person’s diseased liver and replacing it with a healthy one. Liver donations can come from a deceased donor or from a living donor.

A person receiving a transplant from a living donor receives part of the donor’s liver, which will eventually grow to full size.

What is Stage 4 autoimmune hepatitis?

Stage 4 is liver failure, which means your liver can no longer function or heal itself. In liver failure, the liver can no longer process toxins or drugs, and they build up in your body. Symptoms grow worse and can include mental and physical impairment, appetite and weight loss, diarrhea, and other problems.

What foods should you avoid with autoimmune hepatitis?

In other words, a generally healthy diet that's low in sugar, carbohydrates, red meat, and fried foods can't cure autoimmune hepatitis, but it can help reduce the risk that you also develop cirrhosis or other liver disease.

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