Autoimmune Hepatitis
Explanation | Causes | Symptoms l Diagnosis l Treatment l Prognosis
What is autoimmune hepatitis?
Autoimmune hepatitis is a condition in which a person's own immune system attacks the liver, causing inflammation and liver cell death. The inflammation continues and gets worse over time. If not treated, this can lead to irreversible 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 I or II.
Type I is the most common form in North America. It occurs at any age and is more common in females than males. About half of those with type I have other autoimmune disorders such as thyroidosis, Graves' disease, Sjogren's syndrome, autoimmune anemia and ulcerative colitis.
Type II is less common, and often affects girls ages 2 to 14.
Back to Top
What are the causes of autoimmune hepatitis?
When the immune system is working correctly, it protects the body from infections caused by bacteria and viruses. In the case of an autoimmune disease, the body does not recognize certain cells and body parts as part of itself. The body then goes to war against 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 caused by a virus or bacteria, so it is not a contagious disease. In fact, it is not known what triggers the immune system to react against the liver.
Back to Top
What are signs and symptoms of autoimmune hepatitis?
Fatigue (when you feel tired all the time) is one of the most common symptoms of autoimmune hepatitis. Often when patients experience fatigue along with some of the disease's other symptoms -- including abdominal pain and aching joints -- the cause is mistaken for a mild case of the flu.
Other symptoms may include:
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.
Back to Top
How is it autoimmune hepatitis diagnosed?
Your child's doctor will make a diagnosis based on their symptoms, blood tests and liver biopsy.
A routine blood test for liver enzymes can reveal a pattern typical of hepatitis, but further tests, especially for autoantibodies, are needed to diagnose autoimmune hepatitis.
Antibodies are proteins made by the immune system to fight off bacteria and viruses. In autoimmune hepatitis, two antibodies that may develop in the blood are the ANA (antinuclear antibody) and the SMA (smooth muscle antibodies). Also, gamma globulin, a certain type of blood protein, will be elevated when autoimmune hepatitis is present.
A liver biopsy is needed to determine how much inflammation and scarring has developed.
Back to Top
How is it autoimmune hepatitis treated?
With the right treatment, autoimmune hepatitis usually can be controlled. In fact, recent studies show that continued treatment not only stops the disease from getting worse, but it may actually reverse some of the damage.
Medicine. The primary treatment is medicine to suppress (or slow down) an overactive immune system. Both types of 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.
Another medication, azathioprine, is also used to treat this disease. Like prednisone, 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 monitor your child's condition and report any new symptoms to the doctor because 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, 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 percent, with a good quality of life after recovery.
Back to Top
What is the long-term prognosis?
The outlook for children with autoimmune hepatitis is generally favorable. In about seven out of ten 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.
Back to Top
Contact us
For additional information on this or any Health Topic, please call the Family Resource Center, 513-636-7606, or your pediatrician.
Back to Top
Written 4/03, rev. 9/04, rev. 7/06
