Drawing of a torso showing the digestive system, with the liver
shaded and labeled.
Autoimmune hepatitis affects the liver.
Autoimmune hepatitis is a disease in which the body’s immune
system attacks liver cells. This immune response causes
inflammation of the liver, also called hepatitis. Researchers
think a genetic factor may make some people more susceptible to
autoimmune diseases. About 70 percent of those with autoimmune
hepatitis are female.
The disease is usually quite serious and, if not treated, gets
worse over time. Autoimmune hepatitis is typically chronic,
meaning it can last for years, and can lead to cirrhosis—scarring
and hardening—of the liver. Eventually, liver failure can result.
Autoimmune hepatitis is classified as type 1 or type 2. Type 1 is
the most common form in North America. It can occur at any age but
most often starts in adolescence or young adulthood. About half of
those with type 1 have other autoimmune disorders, such as
* type 1 diabetes
* proliferative glomerulonephritis, an inflammation of blood
vessels in the kidneys
* thyroiditis, an inflammation of the thyroid gland
* Graves’ disease, the leading cause of overactive thyroid
* Sjögren’s syndrome, a syndrome that causes dry eyes and mouth
autoimmune anemia
* ulcerative colitis, an inflammation of the colon and rectum
leading to ulcers
Type 2 autoimmune hepatitis is less common, typically affecting
girls aged 2 to 14, although adults can have it too.
What is autoimmune disease?
One job of the immune system is to protect the body from viruses,
bacteria, and other living organisms. The immune system usually
does not react against the body’s own cells. However, sometimes it
attacks the cells it is supposed to protect; this response is
called autoimmunity. Researchers think certain bacteria, viruses,
toxins, and drugs trigger an autoimmune response in people who are
genetically susceptible to developing an autoimmune disorder.
What are the symptoms of autoimmune
hepatitis?
Fatigue is probably the most common symptom of autoimmune
hepatitis. Other symptoms include
* an enlarged liver
* jaundice
* itching
* skin rashes
* joint pain
* abdominal discomfort
* spider angiomas, or abnormal blood vessels, on the skin
* nausea
* vomiting
* loss of appetite
* dark urine
* pale or gray-colored stools
People in advanced stages of the disease are more likely to have
symptoms related to chronic liver disease, such as fluid in the
abdomen—also called ascites—and 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—have the same symptoms as autoimmune
hepatitis, tests may be needed for an exact diagnosis. Doctors
should also review and rule out all medicines a patient is taking
before diagnosing autoimmune hepatitis.
How is autoimmune hepatitis diagnosed?
The doctor will make a diagnosis based on symptoms, blood tests,
and a 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. Autoantibodies attack the body’s
cells. In autoimmune hepatitis, the immune system makes one or
more types of autoantibodies. The most common are antinuclear
antibodies (ANA), smooth muscle antibodies (SMA), and antibodies
to liver and kidney microsomes (anti-LKM). People with type 1 have
ANA, SMA, or both, and people with type 2 have anti-LKM.
Blood tests also help distinguish autoimmune hepatitis from other
diseases that resemble it, such as viral hepatitis B or C or a
metabolic disease such as Wilson disease.
Liver biopsy. A tiny sample of liver tissue, examined with a
microscope, can help doctors accurately diagnose autoimmune
hepatitis and tell how serious it is. This procedure is done in a
hospital or outpatient surgical facility.
How is autoimmune hepatitis treated?
Treatment works best when autoimmune hepatitis is diagnosed early.
With proper treatment, autoimmune hepatitis can usually be
controlled. In fact, studies show that sustained response to
treatment stops the disease from getting worse and may reverse
some of the damage.
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 corticosteroid called prednisone. Treatment may begin with a
high dose of 30 to 60 mg per day and be lowered to 10 to 20 mg per
day as the disease is controlled. The goal is to find the lowest
possible dose that will control the disease.
Another medicine, azathioprine (Imuran) is also used to treat
autoimmune hepatitis. Like prednisone, azathioprine suppresses the
immune system, but in a different way. Treatment may begin with
both azathioprine and prednisone, or azathioprine may be added
later, once the disease is under control. The use of azathioprine
allows for a lower dose of prednisone, which in turn reduces
predisone’s side effects.
In about seven out of 10 people, the disease goes into remission
within 3 years of starting treatment. Remission occurs when
symptoms disappear and lab tests show improvement in liver
function. Some people can eventually stop treatment, although many
will see the disease return. People who stop treatment must
carefully monitor their condition and promptly report any new
symptoms to their doctor. Treatment with low doses of prednisone
or azathioprine may be necessary on and off for years, if not for
life.
Some people with mild forms of the disease may not need to take
medication. Doctors assess each patient individually to determine
whether those with mild autoimmune hepatitis should undergo
treatment.
What are the side effects of prednisone
and azathioprine?
Both prednisone and azathioprine have side effects. Because high
doses of prednisone are often 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, a condition called osteoporosis
* thinning of the hair and skin
* diabetes
* high blood pressure
* cataracts
* glaucoma
Azathioprine can lower white blood cell counts and sometimes
causes nausea and poor appetite. Rare side effects are allergic
reaction, liver damage, and pancreatitis, which is an inflammation
of the pancreas gland with severe stomach pain.
Are other treatments for autoimmune
hepatitis available?
People who do not respond to standard immune therapy or who have
severe side effects may benefit from other immunosuppressive
agents such as mycophenylate mofetil, cyclosporine, or tacrolimus.
People who progress to end-stage liver disease—also called liver
failure—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.
Points to Remember
* Autoimmune hepatitis is a long-term disease in which the 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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