What Is Autoimmune Hepatitis?

Autoimmune hepatitis is a chronic condition that occurs when your body’s immune system attacks your liver. The liver is a major organ located on the right side of your abdomen. It helps you digest food and filter waste from the body.

When the immune system attacks the liver cells, the liver becomes inflamed and liver damage can occur. The term hepatitis refers to inflammation and swelling of the liver. When left untreated, autoimmune hepatitis can lead to cirrhosis, chronic liver damage leading to scarring of the liver and liver failure.

Fortunately, autoimmune hepatitis is treatable with corticosteroids and outcomes are good in patients who seek early treatment. 

Types of Autoimmune Hepatitis

There are two types of autoimmune hepatitis. Type 1 autoimmune hepatitis is the most common kind in the United States, while type 2 is more commonly seen in Europe and tends to be a more severe disease. The two forms of autoimmune hepatitis are characterized by the presence of different types of antibodies, proteins released by the immune system to fight bacteria and viruses. They are: 

• Type 1 is the most common, accounting for 96% of autoimmune hepatitis cases in North America. It usually affects young women who have other autoimmune conditions, such as type 1 diabetes, thyroiditis, and celiac disease. People with this type of autoimmune hepatitis have antinuclear antibodies (ANA) and anti–smooth muscle antibody (ASMA).
• Type 2 is less common in North America, making up only 4% of all autoimmune hepatitis cases. It typically affects females ages 2–14. Individuals with this type of autoimmune hepatitis have anti-liver kidney microsomal antibody type 1 (anti-LKM1) and/or anti-liver cytosol type 1 (anti-LC1) autoantibodies.

Autoimmune Hepatitis Symptoms

Autoimmune hepatitis symptoms vary from person to person. You may first notice a feeling of extreme tiredness or fatigue. Other common symptoms include:

• Abdominal pain or discomfort
• Achy joints
• Itching
• Nausea
• Loss of appetite 

Autoimmune hepatitis causes your liver to become enlarged. You may notice pain or discomfort on the right side of your abdomen just below your ribs. Jaundice is also a common symptom of hepatitis. This results in a yellow tint to the skin and yellowing of the whites of the eyes caused by excess bilirubin pigment. 

Less common symptoms of autoimmune hepatitis include:

• Dark urine
• Pale (clay-colored) stools
• Absence of menstruation (in girls and women)
• Mental confusion
• Fluid buildup in the abdomen, known as ascites

Causes

Autoimmune hepatitis occurs when the immune system attacks the liver cells, causing inflammation and long-term damage. The exact cause is unknown, but it's believed that a combination of genetic and environmental factors can lead to this condition.

In fact, researchers believe that autoimmune hepatitis can be triggered by environmental factors in people who are already genetically predisposed to the condition. About 70% of those diagnosed with autoimmune hepatitis are women ages 15–40.

Individuals who have the following genes are more likely to be diagnosed with autoimmune hepatitis:

• HLA DRB1*03
• HLA DRB1*04

Autoimmune hepatitis is also associated with other autoimmune conditions. If you have any of the following chronic conditions, you may be at higher risk of contracting autoimmune hepatitis:

• Autoimmune thyroiditis: Also known as Hashimoto's disease, autoimmune thyroiditis occurs when the body's immune system attacks the thyroid.
• Grave's disease: Grave's disease causes overactive thyroid, known as hyperthyroidism.
• Ulcerative colitis: Ulcerative colitis is an inflammatory bowel disease that affects the digestive tract.
• Vitiligo: Vitiligo causes a loss of pigment or color in the skin.
• Rheumatoid arthritis: Rheumatoid arthritis is an autoimmune disease that causes the immune system to attack tissues in the body.
• Scleroderma: Scleroderma is an autoimmune disease that causes the skin to tighten and harden.
• Inflammatory bowel disease: Inflammatory bowel disease causes diarrhea, abdominal pain, and an urgency to empty the bowels.
• Sjögren syndrome: Sjogren's syndrome is an autoimmune disease that causes joint pain and other symptoms throughout the body.
• Systemic lupus erythematosus: Lupus is an autoimmune disease that causes the immune system to attack healthy tissues in the body.

Autoimmune hepatitis can also be triggered by prescription drugs. Medications that have been associated with autoimmune hepatitis include:

• Macrobid (nitrofurantoin)
• Dynacin (minocycline)
• Fluthan (halothane)
• Caduet (atorvastatin)
• IsonaRif (isoniazid)
• Voltaren (diclofenac)
• Propycil (propylthiouracil)
• Remicade (infliximab)

Diagnosis

Autoimmune hepatitis is usually diagnosed by physical exam, a detailed medical history, and blood tests. Your doctor may recommend a liver biopsy (removing a small sample of the liver and examining it) to confirm the diagnosis as well. 

Blood tests used to diagnose autoimmune hepatitis include:

• Liver enzymes ALT and AST (elevated levels indicate inflammation in the liver)
• Liver function tests, including bilirubin, cholinesterase, and thrombocytes 
• Serum IgG and other gamma globulins (elevated levels without the presence of cirrhosis can indicate autoimmune hepatitis) 
• Autoimmune antibodies such as anti-LKM-1 and SMA

You may also have imaging tests such as:

• CT (computed tomography) scan
• MRI (magnetic resonance imaging)
• Ultrasound

Treatment

The goal of autoimmune hepatitis treatment is to control the disease and alleviate symptoms. The steroid prednisone is usually prescribed to suppress the immune system’s response and protect the liver.

Your doctor may also recommend a second medication called Imuran (azathioprine). You will likely start by taking a high dose of prednisone and then slowly wean off it as symptoms improve. 

Autoimmune hepatitis can be controlled but not cured. Treatment may include years of steroid use. It is possible that you may require medication for the rest of your life. 

Possible side effects of long-term steroid use include:

• Diabetes (high blood sugar levels)
• Osteoporosis (weak bones)
• Hypertension (high blood pressure)
• Glaucoma (damage to the optic nerve)
• Weight gain
• Increased risk of infections
• Emotional instability 

Because of the risk of osteoporosis with steroid use, your doctor may recommend taking a calcium and vitamin D supplement to protect your bones. 

Prognosis 

Autoimmune hepatitis is a chronic condition that may require lifelong treatment. Once you start steroid therapy to suppress your immune system’s attack on the liver, it can take at least six months to two years to achieve healthy liver enzyme levels.

It is possible to go into remission from autoimmune hepatitis. Once your blood tests have reached normal levels, your medical team will work with you to develop a management plan. It is possible for some individuals to discontinue their steroid medication. However, most people require lifelong treatment. 

Once you have started treatment, it can take six months to a few years for the disease to go into remission. Some people can stop taking their medicine, but often the disease comes back. Some people need to remain on treatment if they have relapsed many times or if their disease is severe. In some cases, autoimmune hepatitis may go away without taking any medicine. But for most people, autoimmune hepatitis is a chronic disease.

If steroid therapy is not effective for you, it is possible for autoimmune hepatitis to progress to cirrhosis. This could mean that you will need a liver transplant at some point. About 4% of liver transplants in the United States and Europe are used to treat autoimmune hepatitis. 

For individuals who respond positively to steroid treatment, the 10-year survival rate is 83.8%–94%. Without any treatment, 40%–50% of individuals with severe autoimmune hepatitis will die within six months to five years.

Coping 

Finding ways to cope with the physical symptoms and stress is an important part of your treatment. Studies have shown that not following your treatment plan is associated with a lower quality of life.

Ask questions at your medical appointments to make sure you understand your treatment plan. If you are unable to adhere to your plan or are experiencing side effects, talk with your medical team before stopping treatment. 

It is common to experience anxiety and depression with autoimmune hepatitis, so finding coping strategies to help improve your quality of life is crucial. Ask your doctor for a referral to a mental health provider to help you manage the stress of living with autoimmune hepatitis. An in-person support group or online support community may be helpful as well.

Summary

Autoimmune hepatitis occurs when your immune system attacks healthy liver cells by mistake. It can cause a wide variety of symptoms, including fatigue, joint pain, nausea, itching, and loss of appetite.

It's not clear what causes autoimmune hepatitis, but many researchers believe it's due to a combination of genetic and environmental factors. People with an autoimmune disease may be more likely to develop autoimmune hepatitis. Certain medications can contribute to the condition as well.

A Word From Verywell

Hearing that you have autoimmune hepatitis can be an overwhelming experience, and you may be feeling sad, frustrated, and anxious right now. It is helpful to remember that early diagnosis and treatment are important to achieving a good prognosis.

If you have noticed symptoms such as fatigue or yellowing skin, see your doctor right away. Your medical team will likely recommend blood tests and a liver biopsy to diagnose the condition. Once the diagnosis is confirmed, they will then discuss steroid therapy for treatment. While autoimmune hepatitis often requires lifetime treatment, it is still possible to live a full, healthy life with this condition.

Frequently Asked Questions 

What is it like to live with autoimmune hepatitis?

Autoimmune hepatitis is a chronic condition that may require treatment for the rest of your life. Once your liver function blood tests have returned to a healthy range, your medical team may be able to reduce your steroid use. Most individuals who are treated for autoimmune hepatitis have a good prognosis and do not experience additional complications. While you may need to take a medication every day, you can still go on to live a full, healthy life. 

Who is at risk of getting autoimmune hepatitis?

Women are at higher risk for autoimmune hepatitis than men. Having another autoimmune disease can raise your risk of autoimmune hepatitis, such as type 1 diabetes, thyroiditis, and rheumatoid arthritis.

Do patients recover from autoimmune hepatitis treatment?

Autoimmune hepatitis is a chronic condition, and while it can be managed, it usually cannot be cured. It is possible to achieve remission from autoimmune hepatitis with the use of steroids. However, most individuals will require treatment for the rest of their lives. The 10-year survival rate for people being treated for autoimmune hepatitis is between 83.8%–94%. Without treatment, the survival rate falls to 50%–60%.

What do researchers know about what causes autoimmune hepatitis?

Autoimmune hepatitis is believed to be caused by a combination of genetic and environmental factors. The condition can be triggered by an environmental factor when you are already genetically predisposed to it. The genes HLA DRB1*03 and HLA DRB1*04 have both been linked to an increased risk of autoimmune hepatitis. Autoimmune hepatitis can also be triggered by certain medications or other diseases.