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
Sjogren'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.
Reprinted from National Digestive Diseases Information Clearinghouse.
For More Information
American Association for the Study of Liver Diseases
1001 North Fairfax, Suite 400
Alexandria, VA 22314
Phone: 703-299-9766
Fax: 703-299-9622
Email: aasld@aasld.org
Internet: www.aasld.org
American Liver Foundation
75 Maiden Lane, Suite 603
New York, NY 10038-4810
Phone: 1-800-GO-LIVER (465-4837), 1-888-4HEP-USA (443-7872), or 212-668-1000
Fax: 212-483-8179
Email: info@liverfoundation.org
Internet: www.liverfoundation.org