Autoimmune Hepatitis (AIH) is a chronic liver disease that causes the body’s immune system to mistakenly attack the liver. This causes inflammation, joint pain, and liver damage, which can lead to liver failure. While it does not currently have a cure, there are different ways to treat autoimmune hepatitis with a high rate of remission.
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What Is Autoimmune Hepatitis?
Autoimmune hepatitis (AIH) is a type of chronic liver inflammation. AIH occurs when your body's own immune system mistakenly attacks the liver, causing swelling and damage. Without proper treatment, AIH can become severe and lead to complications such as cirrhosis, or scarring, and liver failure, which requires a liver transplant.
Many types of hepatitis are caused by viruses, but AIH is different. Because it is an autoimmune disease, doctors aren’t certain of the root cause.
People who have AIH sometimes have other autoimmune diseases too, such as scleroderma or type 1 diabetes.
What are the types of autoimmune hepatitis?
There are two types of AIH, depending on the autoantibodies created:
- Type 1, or classic – The most common form of AIH, affecting people of any age or gender.
- Type 2 –The rarer and more severe form of AIH, mostly affecting children (commonly girls between the ages of 2 and 14).
How common is autoimmune hepatitis?
While researchers can't say for sure how many people have AIH, we do know that it is up to four times more common in girls and women than in boys and men.
What causes autoimmune hepatitis?
Doctors don't always know why AIH starts.
Sometimes, parents pass genes with mutations, or changes, to their children. Researchers suspect some environmental factors might cause the immune system to attack the liver in people with these gene changes.
Some infections or medicines might trigger AIH. Viruses might also play a role.
Autoimmune hepatitis risk factors
You are at a higher risk for autoimmune hepatitis if you:
- Have a family history of autoimmune hepatitis.
- Have a history of infections — bacterial or viral.
- Use certain medications, such as minocycline or nitrofurantoin.
- Were born with female sex organs.
Complications of autoimmune hepatitis
Complications from AIH include:
- Acute liver failure – Occurs when your liver begins to rapidly decline or fail without warning.
- Cirrhosis – Happens when scar tissue blocks the flow of blood and prevents the liver from functioning normally. This can lead to liver failure.
- Liver cancer – Cirrhosis in the liver increases your risk for liver cancer. Finding liver cancer early helps improve the diagnosis.
- Liver failure – Also called end-stage liver disease, liver failure occurs when the liver stops functioning and may require a liver transplant.
AIH is a serious health condition that requires immediate medical attention. It is important to talk with your doctor right away if you think you may have autoimmune hepatitis.
What Are the Signs and Symptoms of Autoimmune Hepatitis?
Not everyone who has AIH has symptoms. You might not know you have the disease until liver damage gets worse, or during routine blood tests.
If you do have AIH symptoms, they may include:
- A decrease in appetite.
- Dark urine.
- Fatigue, or feeling extremely tired.
- Jaundice (yellow skin and eyes).
- Light-colored stools.
- Nausea.
- Pain in your joints.
- Pain in your stomach.
- Skin changes, such as acne or rash.
Other people do not present any symptoms until their AIH has progressed into cirrhosis of the liver.
Cirrhosis symptoms include:
- Confusion or difficulty thinking.
- Losing significant weight quickly.
- Itchy skin.
- Swelling of the abdomen.
- Swelling of the legs, ankles, or feet.
- Vomiting blood.
If you are experiencing any symptoms of cirrhosis, call your doctor immediately to receive appropriate help before your liver damage worsens.
How Do You Diagnose Autoimmune Hepatitis?
Doctors often find AIH when testing blood for other conditions they think a person might have.
If you see your doctor for AIH, they will most likely begin by asking you about your medical history and any symptoms you’ve been experiencing. They may ask about medications, supplements, and any other health products you consume. They may ask you about your habits in drinking alcohol. They may ask other specific questions to rule out other autoimmune diseases.
From there, your doctor may perform a physical examination to look for signs of liver damage, which can look like:
- Changes in the skin.
- Enlarged liver or spleen.
- Swelling in abdomen.
- Swelling in legs, ankles, or feet.
- Yellowish hue to eyes.
If your doctor suspects AIH, they may order a variety of tests or imaging to make the diagnosis. In most cases, just one test will not diagnose AIH, so you can expect a combination of diagnostic tests, including a liver biopsy, to make a formal diagnosis.
Here’s what you need to know about these tests:
- Antibody tests – These tests are checking for autoantibodies—which are antibodies that mistakenly attack healthy tissues and cells—which can help diagnose AIH.
- Blood tests – Your doctor may order one or several blood tests to check for signs of AIH or other liver diseases, such as viral hepatitis or Wilson disease.
- Imaging tests – Your doctor may order an ultrasound, CT scan, or MRI to get a better look at your liver and abdomen. These tests can show abnormalities in the liver size, shape, texture, as well as any liver cirrhosis (scarring).
- Liver tests – These tests check liver enzymes alanine aminotransferase (ALT) and aspartate aminotransferase (AST) levels which can determine AIH and show reaction levels to different treatment methods.
In addition to these tests, your doctor will likely order a liver biopsy. During this procedure, a small piece of tissue is taken from your liver to be examined under a microscope. A pathologist will look for damage, scarring, and any other concerning factors that could designate AIH or another liver disease.
How Do You Treat Autoimmune Hepatitis?
- Acne.
- Diabetes.
- Eye problems.
- High blood pressure.
- Mental health issues.
- Osteopenia or loss of bone density.
- Pancreatitis.
- Weight gain.
Side effects of immunosuppressant medications include:
- Liver damage.
- Low white blood count.
- Nausea.
- Skin rash.
- Pancreatitis.
- Vomiting.
Both steroids and immunosuppressants increase your risk for infections and cancer because they drastically decrease your body’s immune system activity. Because of these severe side effects, it is important to follow your doctor’s orders to find the right dosage to relieve your AIH symptoms without causing more complications.
Your doctor will also use continued liver tests to monitor your ALT and AST levels. If your levels start dropping, it means your body is responding to the medication well.
Many people find these medications lower their autoantibodies and relieve their AIH symptoms. The earlier you can catch AIH, the better your chance of experiencing remission and reversing liver damage.
Remission
Remission of AIH can be described as any period of time where you are without symptoms and your test results show a functioning liver. It is common for people with AIH to go into remission after finding the correct treatment. Doctors will prescribe multiple medications until you experience relief.
After two or more years in remission, your doctor may stop your medications altogether to see if you can remain in remission without them. You can continue to expect liver tests to determine your ALT and AST levels and monitor your symptoms.
Unfortunately, since autoimmune hepatitis is a chronic condition, it can return quickly and can become dangerous fast—which is why continued monitoring even in remission is important. Up to 90% of patients with AIH achieve the state of remission at some point.
Lifestyle changes
If you have AIH, it's crucial to take good care of your health.
Your doctor will urge you to:
- Avoid alcohol.
- Exercise.
- Eat a healthy diet.
- Take dietary supplements if needed.
Your doctor may also suggest you meet with a dietitian, depending on the severity of liver damage or cirrhosis.
Surgery for AIH
Rarely, AIH progresses to liver failure or end-stage liver disease. Treatment for liver failure is transplantation surgery to replace your diseased liver with a healthy donated liver. Your donor may be a deceased or living donor.
Organ transplant and replacement surgeries are serious procedures that require time for preparation and recovery.
Why Choose UPMC for Autoimmune Hepatitis Care?
Our liver doctors, or hepatologists, are experts in treating complex liver diseases. We're always researching new treatments and therapies for people with autoimmune AIH.
Our doctors work with you to design a treatment plan to help keep you healthy.
