Autoimmune hepatitis is a condition in which a person's own immune system attacks the liver, causing swelling and liver cell death. The swelling continues and gets worse over time. If not treated, this can lead to permanent cirrhosis (a disease of the liver caused by liver cells that do not work properly), and eventually liver failure.

Autoimmune hepatitis is classified as either type 1 or 2.

Type 1 is the most common form in North America. It occurs at any age and is more common in females.

Type 2 is less common overall, but more common in children. It often affects females ages 2 to 14.

Causes of Autoimmune Hepatitis

When the immune system is working correctly, it protects the body from infections caused by bacteria and viruses. In an autoimmune disease, the body does not recognize certain cells and body parts as part of itself. The body then attacks itself, damaging the body part it thinks is foreign.

When the immune system attacks the liver in this way, it is called autoimmune hepatitis. Autoimmune hepatitis is not contagious. It is not known what triggers the immune system to react against the liver.

Signs and Symptoms of Autoimmune Hepatitis

Fatigue (feeling tired all the time) is one of the most common symptoms of autoimmune hepatitis. Other common symptoms include abdominal pain and aching joints. For this reason, often times, autoimmune hepatitis is mistaken for a virus, as the symptoms are similar to that of such an infection.

Other symptoms of autoimmune hepatitis may include:

• Enlarged liver
• Itching
• Skin rashes
• Dark urine
• Nausea
• Vomiting
• Pale or gray-colored stools
• Loss of appetite

When autoimmune hepatitis progresses to severe cirrhosis, there may be jaundice (yellow coloring to the skin and eyes), swelling of the belly caused by fluid, bleeding in the intestines, or mental confusion. Also, females may stop having their menstrual periods.

Diagnosis of Autoimmune Hepatitis

A doctor will make a diagnosis based on your child’s symptoms, blood tests and liver biopsy.

A routine blood test for liver enzymes can show a pattern typical of hepatitis, but more tests are needed to make a diagnosis. Certain blood tests look for levels of antibodies (proteins that fight off bacteria and viruses) that will be higher in someone with autoimmune hepatitis. A liver biopsy is needed to determine how much swelling and scarring has developed.

Treatment for Autoimmune Hepatitis

With the right treatment, autoimmune hepatitis usually can be controlled. Recent studies show that continued treatment not only stops the disease from getting worse, but it may reverse some of the damage.

Medicine

Medicine helps to slow down an overactive immune system. Both type 1 and type 2 autoimmune hepatitis are treated with daily doses of a steroid called prednisone.

Your child's doctor may start your child on a high dose and lower the dose as the disease is controlled. The goal is to find the lowest possible dose that will control your child's disease.

Patients who have no liver scarring and a mild case of autoimmune disease may be given budesonide. This drug is a form of steroid. It does not have the same level of side effects as prednisone.

Another medication, azathioprine, is also used to treat this disease. Like prednisone and budesonide, azathioprine slows down the immune system, but in a different way.

Treatment with azathioprine helps lower the dose of prednisone needed, thereby reducing steroid side effects. Your doctor may prescribe azathioprine in addition to prednisone once the disease is under control.

Most people with autoimmune hepatitis will need to take prednisone, with or without azathioprine, for years. Some people take it for life. These steroids may slow down the disease, but everyone is different.

In about one out of every four people, treatment eventually can be stopped. However, it is important to carefully watch your child's condition and report any new symptoms to the doctor. The disease may return and be even more severe, especially during the first few months after stopping treatment.

Side Effects

Both prednisone and azathioprine have side effects. Prednisone can cause fluid retention, high blood pressure, weight gain and the face to swell. Azathioprine can lower your child's white blood count and sometimes cause nausea and poor appetite.

Liver Transplantation

People who progress to end stage liver disease (liver failure) may need a liver transplant. The outcome for patients with autoimmune hepatitis is excellent. Survival rates at transplant centers for this condition are well over 90%, with a good quality of life after recovery.

Long-Term Outlook

The outlook for children with autoimmune hepatitis is generally favorable. In about seven out of 10 people, the disease goes into remission, with symptoms becoming less severe within two years of starting treatment.

However, some people whose disease goes into remission will see it return within three years, so treatment may be necessary on and off for years, if not for life.