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

When to see a doctor

Make an appointment with your doctor if you have any signs or symptoms that worry you.

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

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.

Diagnosis

Tests and procedures used to diagnose autoimmune hepatitis include:

• Blood tests. Testing a sample of your blood for antibodies can distinguish autoimmune hepatitis from viral hepatitis and other conditions with similar symptoms. Antibody tests also help pinpoint the type of autoimmune hepatitis you have.
• Liver biopsy. Doctors perform a liver biopsy to confirm the diagnosis and to determine the degree and type of liver damage. During the procedure, a small amount of liver tissue is removed, using a thin needle that's passed into your liver through a small incision in your skin. The sample is then sent to a laboratory for analysis.

Treatment

Regardless of which type of autoimmune hepatitis you have, the goal of treatment is to slow or stop the immune system attack on your liver. This may help slow the progression of the disease. To meet this goal, you'll need medications that lower immune system activity. The initial treatment is usually prednisone. A second medication, azathioprine (Azasan, Imuran), may be recommended in addition to prednisone.

Prednisone, especially when taken long term, can cause a wide range of serious side effects, including diabetes, thinning bones (osteoporosis), broken bones (osteonecrosis), high blood pressure, cataracts, glaucoma and weight gain.

Doctors typically prescribe prednisone at a high dose for about the first month of treatment. Then, to reduce the risk of side effects, they gradually reduce the dose over the next several months until reaching the lowest possible dose that controls the disease. Adding azathioprine also helps you avoid prednisone side effects.

Although you may experience remission a few years after starting treatment, the disease often returns if the drug is discontinued. Depending on your situation, you may require lifelong treatment.

Liver transplant

When medications don't halt the progress of the disease or you develop irreversible scarring (cirrhosis) or liver failure, the remaining option is a liver transplant.

During a liver transplant, your diseased liver is removed and replaced with a healthy liver from a donor. Liver transplants most often use livers from deceased organ donors. In some cases, a living-donor liver transplant can be used. During a living-donor liver transplant, you receive only a portion of a healthy liver from a living donor. Both livers begin regenerating new cells almost immediately.

Preparing for an appointment

If you have any signs or symptoms that worry you, start by making an appointment with your primary care doctor. If your doctor suspects you may have autoimmune hepatitis, you may be referred to a specialist in liver diseases (hepatologist).

Because appointments can be brief and there's often a lot to discuss, it's a good idea to be prepared for your appointment. Here's some information to help you get ready and know what to expect from your doctor.

What you can do

• Be aware of any pre-appointment restrictions. At the time you make the appointment, be sure to ask if there's anything you need to do in advance, such as restrict your diet.
• Write down any symptoms you're experiencing, including any that may seem unrelated to the reason for which you scheduled the appointment.
• Write down key personal information, including any major stresses or recent life changes.
• Make a list of all medications, vitamins or supplements that you're taking.
• Take a family member or friend along to help you remember everything that was discussed.
• Write down questions to ask your doctor.

For autoimmune hepatitis, some basic questions to ask your doctor include:

• What's the most likely cause of my symptoms?
• Are there any other possible causes?
• What tests do I need to confirm that I have autoimmune hepatitis?
• How severe is the damage to my liver?
• Is my condition likely temporary or chronic?
• What are my treatment options?
• Can treatment cure my autoimmune hepatitis?
• What are the potential side effects of each treatment option?
• How might treatment for autoimmune hepatitis affect the management of my other medical conditions?
• Could any of my medications or habits cause my liver problems or make my liver problems worse?
• Are there any dietary restrictions that I need to follow?
• Should I see a specialist?
• Is there a generic alternative to the medicine you're prescribing me?
• Are there any brochures or other printed material that I can take with me? What websites do you recommend?
• How often will I need follow-up visits?

What to expect from your doctor

Your doctor is likely to ask you a number of questions. Being ready to answer them may reserve time to go over any points you want to spend more time on. Your doctor may ask:

• When did you first begin experiencing symptoms?
• Have your symptoms been continuous, or occasional?
• How severe are your symptoms?
• Does anything seem to improve or worsen your symptoms?
• Are you taking any medicines or treatments for your symptoms?
• Do you have a family history of liver disease?