Autoimmune Hepatitis

Autoimmune hepatitis is a chronic liver disease that occurs when your immune system mistakenly attacks your liver cells. This leads to inflammation of your liver (hepatitis), which can cause serious damage over time.

Normally, your immune system makes and sends out antibodies to fight infections. But this condition is an autoimmune disease. That means your immune system attacks your healthy cells by mistake.

There’s no cure for autoimmune hepatitis. But long-term treatment can help reduce inflammation and prevent complications. Without treatment, the disease can worsen and lead to life-threatening health conditions, like cirrhosis and liver failure.

Autoimmune hepatitis is uncommon. Studies show it affects 10 to 50 out of every 100,000 people.

Types of autoimmune hepatitis

There are two main types of autoimmune hepatitis. The difference has to do with which type of antibodies your immune system produces:

• Type 1: This is the most common type. It can affect anyone, but females aged 40 and younger develop it most often. It involves anti-smooth muscle antibodies (anti-SMA), and sometimes others.
• Type 2: This type is much less common but much more severe. It typically develops during childhood. It involves anti-liver-kidney microsomal-1 (anti-LKM-1) antibodies.

Autoimmune hepatitis symptoms

Not everyone has symptoms with autoimmune hepatitis. You may not know you have it until a healthcare provider notices it on a lab test when they’re checking for other issues. Sometimes, symptoms develop later, after the disease starts to affect your liver function. If you do have symptoms, the first thing you may notice is how tired you feel (fatigue).

Other symptoms may include:

• Yellowing of your skin or eyes (jaundice)
• Itchy skin
• Skin rashes
• Joint pain
• Abdominal pain
• Nausea and vomiting
• Loss of appetite
• Dark-colored pee
• Pale or gray-colored poop

How do you get autoimmune hepatitis?

Researchers don’t know the exact cause of autoimmune hepatitis. But they know it’s an autoimmune disorder. These disorders occur when your immune system mistakes healthy cells as a threat. In autoimmune hepatitis, your immune system produces antibodies that target your liver cells.

Factors that may contribute to autoimmune hepatitis include:

• Genetic predisposition: Certain genes appear to make some people more likely to develop autoimmune disorders.
• Viral infections: You’re more likely to get autoimmune hepatitis if you have a history of Epstein-Barr, herpes, parvovirus B19 or hepatitis A, B or C.
• Environmental triggers: These are toxins or events that stress your immune system. In certain people, these factors may trigger an overactive immune response.
• Certain medications: Some drugs may trigger autoimmune hepatitis, including nitrofurantoin, minocycline and atorvastatin.

Is autoimmune hepatitis contagious?

No. Contagious viruses can cause viral hepatitis and spread. These include hepatitis A, B or C. Previous viral infections can trigger autoimmune hepatitis. But it isn’t a viral infection itself. It can’t spread to other people.

Risk factors

Studies suggest that more than 7 in 10 people with autoimmune hepatitis are female. It most commonly appears between the ages of 15 and 40.

Some people who develop the disease have a preexisting autoimmune disorder. These include:

• Type 1 diabetes
• Ulcerative colitis
• Celiac disease
• Vitiligo
• Sjögren’s syndrome
• Graves’ disease
• Rheumatoid arthritis

Complications of this condition

If autoimmune hepatitis worsens, complications may include:

• Cirrhosis
• Liver failure
• Liver cancer
• Enlarged veins (varices)
• Fluid in your abdomen (ascites)

How doctors diagnose this condition

Your healthcare provider will ask about your symptoms, medical history and any other factors in your life that may cause liver issues. For example, they may ask about how much alcohol you drink. They may also want to review all the prescription and over-the-counter medications you take.

Your provider will also perform a physical exam, checking for signs of liver damage. These signs may include:

• Jaundice
• Enlarged liver or spleen
• Swelling or tenderness in your abdomen
• Swelling in your lower legs, ankles or feet

Tests that are used

Your healthcare provider may refer you to a GI specialist or a liver doctor. This provider will use a combination of tests to make an autoimmune hepatitis diagnosis. These tests may include:

• Blood tests to rule out other causes of liver disease
• Liver function tests to check your liver enzyme levels
• Antibody tests to check for antibodies that mistakenly attack healthy cells
• Imaging tests, including ultrasound, CT scan and MRI scan
• Liver biopsy to confirm a diagnosis and how severe it is

Autoimmune hepatitis treatment

Your healthcare provider will treat autoimmune hepatitis with medications to suppress your immune system and protect your liver. These medications include corticosteroids and immunosuppressants.

Corticosteroids

Corticosteroids reduce inflammation. Your provider will start with a high dose to calm the inflammation in your body. Then, they’ll gradually lower your dose.

Providers prescribe a steroid called prednisone most often. It works well for most people, but it can have side effects. Other options — like budesonide — may have fewer. But it can’t be used in cirrhosis.

Immunosuppressants

Immunosuppressants slow down or stop the autoimmune response in your body. Your provider may prescribe one with the steroid or after you’ve finished it. Common immunosuppressants are azathioprine and CellCept®.

As azathioprine has fewer side effects than steroids, it may work better for long-term maintenance therapy. You may need to take it on and off for life to keep your immune system from becoming overactive again.

How long does the treatment take to work?

The goal of the medications is to make the disease go into remission. That means that all symptoms and signs of the disease are gone. You may have to take the medications for several months to years before this happens.

When you’ve been in remission for at least two years, your healthcare provider may consider taking you off the immunosuppressant. But after you stop treatment, the disease can come back. This is called relapse. About half of all people who go into remission will have a relapse and need to start medication again.

Some people improve with medication, but not enough to go into remission. In this case, your provider may try different medications. Some people don’t respond at all, and the disease continues to worsen. If the disease becomes severe or you develop complications, you may need a liver transplant.

What is the outlook (prognosis) for someone with this condition?

Untreated autoimmune hepatitis can lead to severe complications. Without treatment, about 50% of people die within five years. About 10% of people with untreated autoimmune hepatitis will be alive in 10 years.

With treatment, the prognosis is much better. After treatment, 91% of people with autoimmune hepatitis are alive in 10 years. After 20 years, 70% of people with the condition are still living.