Autoimmune Hepatitis
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Autoimmune hepatitis
affects the liver. |
Autoimmune hepatitis is a disease in which the body's immune
system attacks liver cells. This causes the liver to become inflamed
(hepatitis). Researchers think a genetic factor may predispose some
people to autoimmune diseases. About 70 percent of those with
autoimmune hepatitis are women, most between the ages of 15 and
40.
The disease is usually quite serious and, if not treated, gets
worse over time. It's usually chronic, meaning it can last for
years, and can lead to cirrhosis (scarring and hardening) of the
liver 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 among women than men. About half of those with type I
have other autoimmune disorders, such as type 1 diabetes,
proliferative glomerulonephritis, thyroiditis, Graves' disease,
Sjögren's syndrome, autoimmune anemia, and ulcerative colitis. Type
II autoimmune hepatitis is less common, typically affecting girls
ages 2 to 14, although adults can have it too.
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One job of the immune system is to protect the body from viruses,
bacteria, and other living organisms. Usually, the immune system
does not react against the body's own cells. However, sometimes it
mistakenly attacks the cells it is supposed to protect. This
response is called autoimmunity. Researchers speculate that certain
bacteria, viruses, toxins, and drugs trigger an autoimmune response
in people who are genetically susceptible to developing an
autoimmune disorder.
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Fatigue is probably the most common symptom of autoimmune
hepatitis. Other symptoms include
- enlarged liver
- jaundice
- itching
- skin rashes
- joint pain
- abdominal discomfort
- fatigue
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- spider angiomas (abnormal blood vessels) on the
skin
- nausea
- vomiting
- loss of appetite
- dark urine
- pale or gray colored stools
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People in advanced stages of the disease are more likely to have
symptoms such as fluid in the abdomen (ascites) or mental confusion.
Women may stop having menstrual periods.
Symptoms of autoimmune hepatitis range from mild to severe.
Because severe viral hepatitis or hepatitis caused by a drug--for
example, certain antibiotics--has the same symptoms, tests may be
needed for an exact diagnosis. Your doctor should also review and
rule out all your medicines before diagnosing autoimmune
hepatitis.
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Your doctor will make a diagnosis based on your symptoms, blood
tests, and liver biopsy.
- Blood tests. A routine blood test for liver enzymes can
help 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, the
immune system makes antinuclear antibodies (ANA), antibodies
against smooth muscle cells (SMA), or liver and kidney microsomes
(anti-LKM). The pattern and level of these antibodies help define
the type of autoimmune hepatitis (type I or type II).
Blood
tests also help distinguish autoimmune hepatitis from viral
hepatitis (such as hepatitis B or C) or a metabolic disease (such
as Wilson's disease).
- Liver biopsy. A tiny sample of your liver tissue,
examined under a microscope, can help your doctor accurately
diagnose autoimmune hepatitis and tell how serious it is. You will
go to a hospital or outpatient surgical facility for this
procedure.
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Treatment works best when autoimmune hepatitis is diagnosed
early. With proper treatment, autoimmune hepatitis can usually be
controlled. In fact, recent studies show that sustained response to
treatment not only stops the disease from getting worse, but also
may actually reverse some of the damage.
The primary treatment is medicine to suppress (slow down) an
overactive immune system.
Both types of autoimmune hepatitis are treated with daily doses
of a corticosteroid called prednisone. Your doctor may start you on
a high dose (20 to 60 mg per day) and lower the dose to 5 to 15
mg/day as the disease is controlled. The goal is to find the lowest
possible dose that will control your disease.
Another medicine, azathioprine (Imuran) is also used to treat
autoimmune hepatitis. Like prednisone, azathioprine suppresses the
immune system, but in a different way. It helps lower the dose of
prednisone needed, thereby reducing its side effects. Your doctor
may prescribe azathioprine, in addition to prednisone, once your
disease is under control.
Most people will need to take prednisone, with or without
azathioprine, for years. Some people take it for life.
Corticosteroids may slow down the disease, but everyone is
different. In about one out of every three people, treatment can
eventually be stopped. After stopping, it is important to carefully
monitor your condition and promptly report any new symptoms to your
doctor because the disease may return and be even more severe,
especially during the first few months after stopping treatment.
In about 7 out of 10 people, the disease goes into remission,
with a lessening of severity of symptoms, within 2 years of starting
treatment. A portion of persons with a remission will see the
disease return within 3 years, so treatment may be necessary on and
off for years, if not for life.
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Both prednisone and azathioprine have side effects. Because high
doses of prednisone are needed to control autoimmune hepatitis,
managing side effects is very important. However, most side effects
appear only after a long period of time.
Some possible side effects of prednisone are
- weight gain
- anxiety and confusion
- thinning of the bones (osteoporosis)
- thinning of the hair and skin
- diabetes
- high blood pressure
- cataracts
- glaucoma
Azathioprine can lower your white blood cell count and sometimes
causes nausea and poor appetite. Rare side effects are allergic
reaction, liver damage, and pancreatitis (inflammation of the
pancreas gland with severe stomach pain).
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People who do not respond to standard immune therapy or who have
severe side effects may benefit from other immunosuppressive agents
like mycophenylate mofetil, cyclosporine or tacrolimus. People who
progress to end stage liver disease (liver failure) and/or cirrhosis
may need a liver transplant. Transplantation has a 1-year survival
rate of 90 percent and a 5-year survival rate of 70 to 80
percent.
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Scientists are studying various aspects of autoimmune hepatitis
to find out who gets it and why and to discover better ways to treat
it. Basic research on the immune system will expand knowledge of
autoimmune diseases in general. Epidemiologic research will help
doctors understand what triggers autoimmune hepatitis in some
people. Research on different steroids, alternatives to steroids,
and other immunosuppressants will eventually lead to more effective
treatments.
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- Autoimmune hepatitis is a long-term disease in which your
body's immune system attacks liver cells.
- The disease is diagnosed using various blood tests and a liver
biopsy.
- With proper treatment, autoimmune hepatitis can usually be
controlled. The main treatment is medicine that suppresses the
body's overactive immune system.
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American Liver Foundation (ALF)
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