Autoimmune hepatitis is
inflammation in your liver that occurs when your body's immune system
attacks your liver. Although the cause of autoimmune hepatitis isn't
entirely clear, some diseases, toxins and drugs may trigger autoimmune
hepatitis in susceptible people, especially women.
Untreated autoimmune hepatitis can lead to scarring of the liver
(cirrhosis) and eventually to liver failure. When diagnosed and treated
early, however, autoimmune hepatitis often can be controlled with drugs
that suppress the immune system.
A liver transplant may be an option when autoimmune hepatitis doesn't
respond to drug treatments or when liver disease is advanced.
Signs and symptoms of autoimmune hepatitis can range from minor to
severe and may come on suddenly or develop over time. Some people have
few, if any, problems in the early stages of the disease, whereas others
experience signs and symptoms that may include:
* Abdominal discomfort
* Joint pain
* Itching (pruritus)
* Yellowing of the skin and whites of the eyes (jaundice)
* An enlarged liver
* Abnormal blood vessels on the skin (spider angiomas)
* Nausea and vomiting
When to see a doctor
Make an appointment with your doctor if you have any signs or symptoms that worry you.
Autoimmune hepatitis occurs when the body's immune system, which
ordinarily attacks viruses, bacteria and other pathogens, instead
targets the liver. This attack on your liver can lead to chronic
inflammation and serious damage to liver cells. Just why the body turns
against itself is unclear, but researchers think autoimmune hepatitis
could be caused by an interaction between several risk factors, such as
infections, medications and a genetic predisposition.
Types of autoimmune hepatitis
Doctors have identified two main forms of autoimmune hepatitis:
* Type 1 (classic) autoimmune hepatitis. Often developing suddenly,
this is the most common type of the disease. It can occur at any age.
About half the people with type 1 autoimmune hepatitis have other
autoimmune disorders, such as thyroiditis, rheumatoid arthritis or
* Type 2 autoimmune hepatitis. Although adults can develop type 2
autoimmune hepatitis, it's most common in young girls and often occurs
with other autoimmune problems.
Factors that may increase your risk of autoimmune hepatitis include:
* Being female. Although both men and women can develop autoimmune hepatitis, the disease is far more common in women.
* Age. Type 1 autoimmune hepatitis can occur at any age. Type 2 primarily affects young girls.
* A history of certain infections. Autoimmune hepatitis may develop after a bacterial or viral infection.
* Use of certain medications. Certain medications, such as the
antibiotic minocycline and the cholesterol medication atorvastatin
(Lipitor) have been linked to autoimmune hepatitis.
* Heredity. Evidence suggests that a predisposition to autoimmune hepatitis may run in families.
Diseases that can occur in people with autoimmune hepatitis
Autoimmune hepatitis may be associated with a variety of other autoimmune diseases, including:
* Pernicious anemia. Associated with a number of autoimmune
disorders, pernicious anemia occurs when a lack of vitamin B-12
interferes with your body's ability to form red blood cells.
* Hemolytic anemia. In this type of anemia, your immune system
attacks and breaks down red blood cells faster than your bone marrow can
* Thrombocytopenic purpura. Platelets are blood cells that help your
blood clot. In thrombocytopenic purpura, your immune system attacks and
destroys these cells, leading to easy bruising and bleeding.
* Ulcerative colitis. This inflammatory bowel disease can cause
severe bouts of watery or bloody diarrhea and abdominal pain.
* Autoimmune thyroiditis (Hashimoto's thyroiditis). In this condition, the immune system attacks the thyroid gland.
* Rheumatoid arthritis. Another autoimmune disease, rheumatoid
arthritis occurs when the immune system attacks the lining of your
joints, leading to stiffness, pain, swelling, and sometimes deformity
* Celiac disease. This disease causes an abnormal reaction to
gluten, a protein found in most grains. Eating gluten sets off an immune
response that damages the small intestine.
Complications of liver damage
Autoimmune hepatitis that goes untreated can cause permanent scarring of
the liver tissue (cirrhosis). Complications of cirrhosis include:
* Increased blood pressure in the vein that sends blood into the
liver. Blood from your intestine, spleen and pancreas enters your liver
through a large blood vessel called the portal vein. If scar tissue
blocks normal circulation through your liver, this blood backs up,
leading to increased pressure within the portal vein (portal
* Enlarged veins in your esophagus (esophageal varices). When
circulation through the portal vein is blocked, blood may back up into
other blood vessels — mainly those in your stomach and esophagus. The
blood vessels are thin walled, and because they're filled with more
blood than they're meant to carry, they're likely to bleed. Massive
bleeding in the esophagus from these blood vessels is a life-threatening
emergency that requires immediate medical care.
* Fluid in your abdomen (ascites). Liver disease can cause large
amounts of fluid to accumulate in your abdomen. Ascites can be
uncomfortable and may interfere with breathing and is usually a sign of
* Liver failure. This occurs when extensive damage to liver cells
makes it impossible for your liver to function. At this point, a liver
transplant is the only option.
* Liver cancer. People with cirrhosis have an increased risk of liver cancer.
Preparing for your appointment
If you have any signs or symptoms that worry you, start by making an
appointment with your family doctor. If your doctor suspects you may
have a liver problem, such as autoimmune hepatitis, you may be referred
to a doctor who specializes in liver diseases (hepatologist).
Because appointments can be brief, and because there's often a lot of
ground to cover, it's a good idea to be prepared for your appointment.
Here's some information to help you get ready, and what to expect from
What you can do
* Be aware of any pre-appointment restrictions. At the time you make
the appointment, be sure to ask if there's anything you need to do in
advance, such as restrict your diet.
* Write down any symptoms you're experiencing, including any that
may seem unrelated to the reason for which you scheduled the
* Write down key personal information, including any major stresses or recent life changes.
* Make a list of all medications, as well as any vitamins or supplements, that you're taking.
* Take a family member or friend along. Sometimes it can be
difficult to absorb all the information provided during an appointment.
Someone who accompanies you may remember something that you missed or
* Write down questions to ask your doctor.
Your time with your doctor is limited, so preparing a list of questions
will help you make the most of your appointment. List your questions
from most important to least important in case time runs out. For
autoimmune hepatitis, some basic questions to ask your doctor include:
* Do I have autoimmune hepatitis?
* Could any of my medications cause my liver problems or make my liver problems worse?
* How severe is the damage to my liver?
* What kinds of tests do I need?
* Is my condition likely temporary or chronic?
* What are my treatment options?
* Can treatment cure my autoimmune hepatitis?
* What are the potential side effects of each treatment option?
* I have these other health conditions. How can I best manage them together?
* Are there any restrictions that I need to follow?
* Should I see a specialist? What will that cost, and will my insurance it?
* Is there a generic alternative to the medicine you're prescribing me?
* Are there any brochures or other printed material that I can take with me? What Web sites do you recommend?
* What will determine whether I should plan for a follow-up visit?
In addition to the questions that you've prepared, don't hesitate to ask
questions at any time that you don't understand something.
Tests and diagnosis
Tests and procedures used to diagnose autoimmune hepatitis include:
* Blood tests. Testing a sample of your blood for antibodies can
distinguish autoimmune hepatitis from viral hepatitis and other
disorders with similar symptoms. Antibody tests also help pinpoint the
type of autoimmune hepatitis you have.
* Liver biopsy. Doctors perform a liver biopsy to confirm the
diagnosis and to determine the degree and type of liver damage. During
the procedure, a small amount of liver tissue is removed, using a thin
needle that's passed into your liver through a small incision in your
skin. The sample is then sent to a laboratory for analysis.
Treatments and drugs
The goal in treating autoimmune hepatitis is to slow or stop your body's
immune system from attacking your liver. This may help slow the
progress of the disease.
Medications to control your immune system (immunosuppressants)
Medications used to treat autoimmune hepatitis include:
Prednisone. Doctors usually recommend an initial high dose of the
corticosteroid drug prednisone for people with autoimmune hepatitis. As
soon as signs and symptoms improve, the medication is reduced to the
lowest possible dose that controls the disease. Most people need to
continue taking the prednisone for years and some people for life.
Although you may experience remission a few years after starting
treatment, the disease usually returns when the drug is discontinued.
Prednisone, especially when taken long term, can cause a wide
range of serious side effects, including diabetes, thinning bones
(osteoporosis), broken bones (osteonecrosis), high blood pressure,
glaucoma and weight gain.
* Azathioprine (Imuran). Azathioprine, another immunosuppressant
medication, is sometimes used along with prednisone. Using both
medications may reduce the dosage of prednisone needed, reducing its
side effects. Side effects of azathioprine may include difficulty
fighting infections and nausea. Rare side effects include liver damage,
pancreas inflammation (pancreatitis) and cancer.
* Other immunosuppressants. If you don't respond to prednisone or
azathioprine, your doctor may prescribe stronger immunosuppressants,
such as cyclosporine (Sandimmune) or tacrolimus (Prograf).
When medications don't halt the progress of the disease, or you have or
develop irreversible scarring (cirrhosis) or liver failure, the
remaining option is a liver transplant.
During a liver transplant, your diseased liver is removed and a healthy
liver from a donor is placed in your body. Liver transplants most often
use livers from deceased organ donors. In some cases, a living-donor
liver transplant can be used. During a living-donor liver transplant,
you receive only a portion of a healthy liver from a living donor.