Overview

What is autoimmune hepatitis?

Autoimmune hepatitis is a chronic liver disease that begins with a mistake of your immune system. Your immune system sends antibodies to your liver tissues, causing inflammation (hepatitis). These antibodies are normally supposed to attack infections in your liver tissues. But in autoimmune disease, your immune system mistakenly attacks your own healthy cells instead.

Autoimmune hepatitis causes chronic inflammation in your liver, which may cause serious damage over time. Like other types of chronic hepatitis, autoimmune hepatitis can lead to scarring of your liver tissues (cirrhosis). Medical treatment can help reduce the inflammation and prevent complications from developing. However, in the early stages of the disease, you may not have symptoms.

What are the two main types of autoimmune hepatitis?

The different types of autoimmune hepatitis (AIH) involve different autoantibodies that scientists can recognize on an antibody test. These different antibodies attack different types of cells in your liver.

Type 1

Type 1 AIH, the “classic” type, is also the most common. It makes up about 80% of diagnosed cases, and it can affect anyone. Type 1 involves anti-smooth muscle antibodies (ASMA) that attack the smooth muscle cells in your liver. Type 1 has also been called “lupoid hepatitis” because its clinical symptoms resemble those of systemic lupus erythematosus. An ASMA blood test can help distinguish the two.

Type 2

Type 2 AIH is more rare and often more severe. It tends to appear earlier, usually during childhood, and progresses faster than type 1. Type 2 involves anti-liver-kidney microsome type 1 antibodies (anti-LKM-1), or anti-liver cytosol type 1 antibodies (anti-LC1). Anti-LKM-1 antibodies target a protein in your liver cells called cytochrome P450-2D6 (CYP2D6). Anti-LC1 antibodies are liver antibodies specific to type 2 AIH.

How rare is autoimmune hepatitis?

Autoimmune hepatitis is uncommon and its exact prevalence is unknown. European studies suggest that between .010% and .025% of the European population is affected. It appears to affect all racial and ethnic groups, but research has found it’s more common among Alaska Natives, affecting about .043% of that population. It’s also more common in women and people assigned female at birth (AFAB), by a ratio of 4:1.

Symptoms and Causes

What are the symptoms of autoimmune hepatitis?

Not everyone has symptoms with autoimmune hepatitis. Sometimes symptoms develop later, after the disease has begun to affect your liver function. This causes various side effects in your body.

Some common early symptoms include:

When your liver function begins to deteriorate, bile can build up in your bloodstream. This can cause:

Jaundice (yellowing of skin and eyes).
Dark-colored pee or pale-colored poop.
Pruritus (itchy skin).
Nausea or loss of appetite.

Other late side effects can include:

Spider angiomas.
Enlarged veins in your esophagus (varices).
Easy bruising and bleeding.
Loss of menstrual periods.
Fluid build-up in your abdomen (ascites) or hands and feet (edema).
Confusion, disorientation or drowsiness (hepatic encephalopathy).

What causes autoimmune hepatitis?

Autoimmune diseases occur when your immune system mistakes some of your own cells for a threat. Once your immune system identifies this threat, it continues to attack these cells, causing chronic inflammation in some part of your body. In autoimmune hepatitis, your immune system sends specific antibodies to attack specific types of liver cells.

What is the average age of onset?

It’s not always possible to know when autoimmune hepatitis originally began, since it often doesn’t cause symptoms right away. Most people are diagnosed with type 1 AIH in early to middle adulthood, between the ages of 15 and 40. But it can appear at any age. Type 2 AIH typically appears earlier, between the ages of 4 and 14. It may appear with already advanced symptoms of liver disease.

How do you get autoimmune hepatitis?

Why people get autoimmune diseases is a complicated question. There seem to be multiple factors involved. In many cases, certain genes appear to make you more susceptible to developing certain autoimmune disorders. But not everyone with those genes develops the disease, and not everyone who develops it has those genes. Other, nongenetic factors, called “environmental” factors, also contribute.

Environmental factors are toxins or events that stress your immune system. In certain susceptible individuals, these factors may trigger an overactive immune response that manifests as a chronic autoimmune disease. Triggers linked to autoimmune hepatitis include certain drugs and certain viral infections. You’re also more susceptible if you have another autoimmune disease.

What viruses may trigger autoimmune hepatitis?

You’re more likely to get autoimmune hepatitis if you have a history of:

What drugs can trigger autoimmune hepatitis?

Drug-induced autoimmune hepatitis has been associated with:

Nitrofurantoin (for urinary tract infections).
Minocycline (for acne).
Atorvastatin (for high cholesterol).
Isoniazid (an antibiotic).

What other autoimmune diseases are associated with autoimmune hepatitis?

Some people develop autoimmune hepatitis with another autoimmune disease that affects their bile ducts, such as:

This is considered a variant type of autoimmune hepatitis.

But in general, any preexisting autoimmune disease can make you more likely to develop another one. Chronic inflammation in one area appears to trigger it in another. The chance of developing any second autoimmune disease is 25%-50%. If autoimmune hepatitis is your first autoimmune disease, you also have the same chance of developing a second one.

Other conditions commonly seen with AIH include:

Is autoimmune hepatitis contagious?

No. Contagious viruses can cause viral hepatitis (such as hepatitis A, hepatitis B or hepatitis C). These infections can spread, but autoimmune hepatitis isn’t an infection and can’t spread to other people.

Diagnosis and Tests

How do you diagnose autoimmune hepatitis?

You may or may not have noticeable signs or symptoms of AIH. A healthcare provider will begin by giving you a physical exam and some routine tests, such as imaging and blood tests. A blood test called a comprehensive metabolic panel will show evidence of liver disease. The panel includes a selection of liver function tests that measure liver enzymes and inflammation, among other things.

These indicators aren’t specific to autoimmune hepatitis, though. Your provider will do additional blood work to check for specific viruses and other causes of hepatitis. They’ll also look for autoantibodies. When they’ve ruled out other causes and identified the antibodies associated with autoimmune hepatitis, they’ll be ready to confirm the diagnosis. The best way to do this is with a liver biopsy.

Management and Treatment

What is the treatment for autoimmune hepatitis?

The standard treatment is to begin with a high dose of corticosteroids to calm the inflammation and suppress the autoimmune response, then slowly taper off. Prednisone is the most commonly prescribed and the most studied medication for autoimmune hepatitis. It works well for most people, but it can have side effects. Alternatives, such as budesonide, seem to have fewer.

Your healthcare provider may prescribe an immunosuppressant called azathioprine along with steroids, or they may prescribe it after you’ve finished your course of steroid therapy. Since azathioprine has fewer side effects than steroids, it works better for long-term maintenance therapy, to keep your immune system from becoming overactive again. You may need to take it on and off for life.

What are the side effects of the medication?

Side effects of long-term steroid use can include:

Increased appetite and weight gain.
Mood disorders, such as anxiety and depression.
Glaucoma (vision blurring).
Osteopenia or osteoporosis (bone weakening).
Diabetes.
High blood pressure.

Side effects of taking immunosuppressants can include:

Frequent infections.
Nausea and vomiting.
Skin rashes.
Easy bruising and bleeding.
Impaired kidney function.
Pancreatitis.

When you take these medications, your healthcare provider will monitor you for side effects. If the side effects of your medication are too severe, or it doesn’t help you enough, they’ll suggest an alternative.

How long does the treatment take to work?

The goal of the medications is to make the disease go into remission. You may have to take them for several months to years before this happens. Along the way, your healthcare provider will check your liver regularly for signs the treatment is working. Liver function tests will show your liver enzyme levels gradually lowering to normal. Remission means that all symptoms and signs of the disease are gone.

The American Association for the Study of Liver Diseases recommends staying on steroids for at least three years before discontinuing. When you’ve been in remission for at least two years, they’ll consider discontinuing the immunosuppressants. About 50% of people will have a relapse of the disease within three months of discontinuing their medications. Others may relapse years later, or not at all.

Some people have some improvement with treatment, but not enough to achieve remission. In this case, your healthcare provider will try different medications. Some people don’t respond at all to the treatment. In this case, the course of the disease continues to worsen. These people may develop complications that need additional treatment. They may eventually need a liver transplant.

Outlook / Prognosis

Can autoimmune hepatitis be cured?

No. It can go into remission. That means the inflammatory process goes away for a time, sometimes for a long time. But after you stop the treatment, it can come back. This is called relapse. Most people (80%) who discontinue their medications will eventually have a relapse and need to start them again. Medications can usually control the disease well, but you may have to take them off and on for life.

What is the average life expectancy with autoimmune hepatitis?

Without treatment, life expectancy is 50% within five years. But with treatment, life expectancy is 90% in 10 years and 70% in 20. About 15% of people will eventually develop cirrhosis despite treatment, usually after 10 to 20 years. This can happen if the treatment fails, if you have an incomplete response to the treatment or if the disease relapses multiple times. When it relapses, it can come back stronger.

Living With

How should I take care of myself while living with autoimmune hepatitis?

Keep up with your healthcare appointments. Your healthcare provider will need to continue to monitor your liver for the rest of your life. Even if you’ve been in remission for some time, the disease can relapse without warning and without causing noticeable symptoms. Your healthcare provider can monitor the extent of fibrosis (damage that leads to scarring) in your liver through noninvasive elastography. If you do begin to have symptoms again, don’t hesitate to contact your healthcare provider.
Take care of your diet. A healthy diet is very important for anyone with liver disease. With AIH in particular, studies show that up to 30% of people have signs of non-alcohol related fatty liver disease. That means your body has a tendency to store excess fat in your liver, which is an additional cause of inflammation. You can work against this by maintaining a healthy weight and reducing sugar and saturated fats in your diet. This can also help improve your treatment results.
Protect your immunity. Both liver disease and immunosuppressant drugs depress your immune system. That means you have to take extra good care of yourself to prevent getting sick. Your healthcare provider may recommend certain vitamin supplements and vaccines to protect you against infections. Make sure you don’t take any supplements that they haven’t approved.
Avoid alcohol. Alcohol use depresses your immunity and damages your liver.

A note from Cleveland Clinic

Autoimmune hepatitis is serious, but most people respond well to treatment. The disease can go into remission and all but disappear for a long time. When it returns, the same treatment is likely to work again. There may be side effects to the treatment, but by taking care of your diet and immunity, you can live a relatively normal life. When the treatment doesn’t work, liver transplantation is an option.

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Autoimmune Hepatitis