Autoimmune Hepatitis

Autoimmune hepatitis is a chronic liver disease where your own immune system mistakenly attacks your liver, causing inflammation and potential damage. While the exact cause remains unclear, most people can manage this condition with medication and many achieve remission, allowing them to live normal lives.

Table of contents

• What is autoimmune hepatitis?
• Types of autoimmune hepatitis
• Who gets autoimmune hepatitis?
• Symptoms and warning signs
• What causes autoimmune hepatitis?
• How is it diagnosed?
• Treatment options
• Living with autoimmune hepatitis
• Outlook and prognosis

What is autoimmune hepatitis?

Autoimmune hepatitis is a long-term disease that happens when your body’s immune system—which normally protects you from infections—makes a mistake and attacks your liver instead^[1]. Your immune system produces special proteins called antibodies that are supposed to fight off germs and viruses. In autoimmune hepatitis, these antibodies target your own healthy liver cells, causing swelling and damage^[2].

This attack on the liver causes inflammation, which means swelling and irritation of liver tissue. The inflammation is called hepatitis^[1]. Over time, if the inflammation continues without treatment, it can lead to scarring of the liver tissue, called cirrhosis. In severe cases, this can progress to liver failure^[1].

The good news is that when diagnosed and treated early, autoimmune hepatitis can often be controlled with medicines^[1]. Many people with this condition can achieve remission, which is a period when symptoms disappear and the liver is no longer being damaged^[9]. With proper treatment, most patients lead long lives and have normal life expectancies without needing a liver transplant^[16].

Types of autoimmune hepatitis

Doctors have identified two main types of autoimmune hepatitis based on the different antibodies found in blood tests^[2]. These different antibodies attack different types of cells in the liver.

Type 1 autoimmune hepatitis is the most common form, making up about 80% to 96% of all cases^[2][7]. It can affect people of any age and involves antibodies called anti-smooth muscle antibodies (ASMA) and antinuclear antibodies (ANA)^[2][7]. Type 1 has sometimes been called “lupoid hepatitis” because its symptoms can look similar to those of lupus, another autoimmune disease^[2].

Type 2 autoimmune hepatitis is much less common, accounting for only about 5% to 10% of cases^[3]. This type usually appears during childhood or early teenage years and tends to be more severe than Type 1^[2][7]. It involves different antibodies called anti-liver-kidney microsome type 1 (anti-LKM-1) or anti-liver cytosol type 1 (anti-LC1)^[2][7]. Type 2 often progresses faster and may be more difficult to treat^[7].

Who gets autoimmune hepatitis?

Autoimmune hepatitis is an uncommon condition. Studies suggest that between 4 to 43 out of every 100,000 adults have this disease^[3]. It can affect people of all ages and racial and ethnic backgrounds^[3].

However, certain groups are more likely to develop autoimmune hepatitis. The disease is much more common in women than in men. Studies have found that between 71% and 95% of adults with autoimmune hepatitis are women, and between 60% and 76% of children with the disease are girls^[3]. The ratio of women to men affected is about 4 to 1^[2].

Research has found that autoimmune hepatitis is more common among Alaska Natives, affecting about 0.043% of that population^[2]. The disease can develop at any age, with two peak times when it most often appears: in young adults during their 20s and in middle-aged adults during their 50s^[7].

Some people may be more at risk if they have other autoimmune diseases or a family history of autoimmune conditions^[3]. Genetic factors, particularly certain genes that control immune system function, appear to play a role in who develops the disease^[1].

Symptoms and warning signs

Many people with autoimmune hepatitis have no symptoms at all, especially in the early stages of the disease^[1][2]. In fact, as many as one-third of patients are asymptomatic when diagnosed, with the condition discovered only after blood tests show unexplained elevations in liver enzymes^[6].

When symptoms do occur, they can develop suddenly or gradually over time. Early symptoms are often nonspecific and may include^[1][2]:

• Extreme tiredness or fatigue
• Discomfort or pain in the belly, particularly in the upper right area
• Joint pain
• Skin rashes or acne
• Loss of appetite
• Nausea
• Weight loss
• A swollen abdomen with an enlarged liver

As liver function begins to worsen, a substance called bile can build up in the bloodstream. This causes additional symptoms including^[2]:

• Jaundice—yellowing of the skin and the whites of the eyes (depending on skin color, this change may be harder or easier to see)
• Dark-colored urine (pee)
• Pale-colored stools (poop)
• Severe itching of the skin

In more advanced stages, when the liver has been significantly damaged, people may experience^[1][2]:

• Spider angiomas—small, spider-like blood vessels visible on the skin
• Easy bruising and bleeding
• Fluid buildup in the abdomen (called ascites) or in the hands and feet (called edema)
• Loss of menstrual periods in women
• Confusion, disorientation, or drowsiness (called hepatic encephalopathy)

About 25% of patients experience an acute onset that looks similar to sudden hepatitis from other causes^[6]. If you experience any of these symptoms, especially jaundice or severe fatigue, it’s important to see a healthcare professional promptly^[1].

What causes autoimmune hepatitis?

The exact cause of autoimmune hepatitis remains unclear, but researchers believe it results from a combination of genetic and environmental factors that interact over time^[1].

Your immune system normally protects your body by producing antibodies and special white blood cells called lymphocytes that fight off infections. In autoimmune hepatitis, something causes the immune system to malfunction. It starts producing certain types of antibodies—called autoantibodies—that mistakenly identify your liver cells as a threat and attack them^[3].

Scientists have identified several genetic factors that increase the risk of developing autoimmune hepatitis. The disease is strongly linked to certain genes, particularly those called HLA genes, which help control immune system function. Specific HLA gene variations, such as DRB1*0301 and DRB1*0401, are more common in people with autoimmune hepatitis in North American and European populations^[13].

However, having these genes doesn’t mean you’ll definitely develop the disease. Environmental factors also play an important role. Certain triggers may cause the immune system to start attacking the liver in people who are genetically susceptible. These triggers might include^[1]:

• Viral infections
• Certain medications or chemicals
• Other environmental exposures

Recent research suggests that problems with special immune cells called regulatory T cells (Tregs) may contribute to autoimmune hepatitis^[13]. These cells normally help control the immune system and prevent it from attacking the body’s own tissues. When regulatory T cells don’t work properly, they can’t stop other immune cells from attacking the liver^[11].

The attack on liver cells triggers a complex inflammatory process. Damaged liver cells release more proteins that the immune system recognizes as foreign, which leads to more antibody production and more inflammation. This creates an ongoing cycle of liver damage^[11].

How is it diagnosed?

Diagnosing autoimmune hepatitis involves several steps, as there is no single test that can confirm the condition^[6]. Doctors must rule out other causes of liver disease and look for characteristic patterns in test results.

Blood tests are usually the first step. These tests can show^[1][8]:

• Elevated liver enzymes, particularly AST (aspartate aminotransferase) and ALT (alanine aminotransferase), which indicate liver inflammation
• High levels of a protein called immunoglobulin G (IgG), which is often elevated in autoimmune hepatitis
• Specific autoantibodies in the blood, which help identify the type of autoimmune hepatitis

For suspected Type 1 autoimmune hepatitis in adults, doctors test for antinuclear antibodies (ANA) and anti-smooth muscle antibodies (ASMA)^[7]. For children, or if Type 2 is suspected, additional tests for anti-liver-kidney microsome type 1 (anti-LKM1) antibodies are performed^[7].

A liver biopsy is often needed to confirm the diagnosis^[8]. During this procedure, a doctor inserts a thin needle through the skin and into the liver to remove a small sample of liver tissue. The sample is then examined under a microscope in a laboratory. The biopsy helps doctors see the extent of liver damage and look for characteristic signs of autoimmune hepatitis, such as interface hepatitis with plasma cell infiltration^[7].

Before confirming autoimmune hepatitis, doctors must rule out other conditions that can cause similar symptoms and test results, including^[7]:

• Viral hepatitis (such as hepatitis B or C)
• Drug-induced liver injury
• Metabolic dysfunction-associated steatotic liver disease (MASLD)
• Primary sclerosing cholangitis (PSC)
• Primary biliary cholangitis (PBC)

Sometimes patients have features of more than one liver disease. This is called an “overlap syndrome” or variant form, and it may affect treatment decisions^[6].

Treatment options

The main goal of treating autoimmune hepatitis is to slow or stop the immune system’s attack on the liver, which helps reduce inflammation and prevent complications^[8]. Treatment usually involves medications that suppress the immune system, called immunosuppressants.

Standard treatment typically begins with a corticosteroid medication called prednisone^[8][9]. Doctors usually start with a relatively high dose for about the first month to control the inflammation quickly. Then they gradually reduce the dose over several months to minimize side effects while maintaining control of the disease^[8].

Because prednisone can cause serious side effects when taken long-term—including diabetes, weakened bones, high blood pressure, cataracts, glaucoma, and weight gain—doctors often add a second medication called azathioprine (Azasan or Imuran)^[8][9]. Adding azathioprine allows doctors to lower the prednisone dose while still controlling the liver inflammation. Some patients may eventually take only azathioprine for maintenance therapy^[9].

Before starting azathioprine, many doctors recommend a genetic test to check for variations in an enzyme called TPMT (thiopurine methyltransferase)^[14]. People with certain TPMT variations may be at higher risk for serious side effects from azathioprine, including bone marrow suppression.

For patients who don’t respond well to standard treatment or can’t tolerate these medications, other immunosuppressants may be tried, such as 6-mercaptopurine or additional medications^[9].

Achieving remission is an important goal of treatment. With proper medication, many people achieve remission, meaning their symptoms disappear, liver enzyme levels return to normal, and the liver is no longer being damaged^[9]. Some people stay in remission for at least two years, at which point doctors may try to gradually reduce or stop medications to see if the remission continues without them^[9].

However, autoimmune hepatitis is often a lifelong condition. Many patients experience a relapse—meaning the disease becomes active again—if medications are stopped^[9]. When this happens, treatment must be restarted. Some people need to stay on low-dose maintenance therapy indefinitely to keep the disease under control.

Regular monitoring with blood tests is essential during and after treatment to check liver enzyme levels and watch for relapses^[9]. Your doctor will continue to perform routine blood tests even when you’re in remission.

In cases where the disease has progressed to advanced liver failure and doesn’t respond to medications, a liver transplant may be an option^[1][8]. After a liver transplant, it’s possible for autoimmune hepatitis to come back in the new liver, but transplantation can be life-saving for people with end-stage liver disease.

Not everyone diagnosed with autoimmune hepatitis needs to start treatment immediately. Patients with very mild disease who aren’t having symptoms may be able to delay treatment under close medical supervision^[14]. However, this decision must be made carefully with your doctor, as untreated disease can progress rapidly.

Living with autoimmune hepatitis

Living with autoimmune hepatitis requires ongoing medical care, but many people with this condition lead full and active lives^[16].

Regular medical follow-up is essential. You’ll need frequent appointments with your doctor or liver specialist to monitor your condition with blood tests and adjust medications as needed^[15]. The frequency of these appointments will depend on how well controlled your disease is.

Taking medications consistently is crucial for keeping the disease under control. Missing doses can lead to flare-ups of liver inflammation^[15]. If you have trouble remembering to take your medications, talk to your doctor about strategies that can help, such as using pill organizers or setting phone reminders^[21].

Some patients experience what’s often called a “flare”—an increase in liver enzyme levels even while on medication^[16]. Flares may occur for various reasons, including not taking medication consistently, stress, viral illnesses, or other liver conditions developing. During a flare, you might notice symptoms like worsening fatigue, itching, or joint pain returning. Report any significant changes in symptoms to your doctor promptly^[16].

Lifestyle considerations are also important. Avoid alcohol completely, as it can make liver damage worse^[19]. Make sure all your doctors know about your condition and all the medications you take. Some medicines, including over-the-counter drugs like acetaminophen (Tylenol), can harm the liver and should only be used under medical supervision^[19].

Your doctor may recommend a low-salt diet, especially if you develop fluid retention^[19]. Salt is found in many prepared and packaged foods, so look for reduced-salt products. If you experience itchy skin, a common symptom, keeping cool, staying out of the sun, and wearing cotton clothing may help^[19].

Match your activity level to your energy^[19]. Fatigue is common, so it’s important to rest when needed. However, staying physically active within your limits can be beneficial for overall health.

Pregnancy considerations are important for women of childbearing age. Many women with well-controlled autoimmune hepatitis can have successful pregnancies, but special care and close monitoring are essential^[7]. Some medications used to treat autoimmune hepatitis may need to be adjusted during pregnancy. Discuss your plans with your doctor before becoming pregnant.

Emotional support is valuable when living with a chronic condition. Connecting with others who understand what you’re going through can be helpful. Support groups, both in-person and online, allow you to share experiences and coping strategies^[20][21]. Organizations like the Autoimmune Hepatitis Association offer resources, webinars, and connections to other patients.

Mental health is important too. Living with autoimmune hepatitis can affect your emotional well-being. Don’t hesitate to seek help from a counselor or therapist if you’re struggling with anxiety, depression, or the stress of managing a chronic illness^[21].

Outlook and prognosis

The outlook for people with autoimmune hepatitis has improved dramatically with modern treatment. When properly treated, most people with this condition can expect to live long lives with normal or nearly normal life expectancy^[6][16].

Studies show that patients being treated for autoimmune hepatitis have good long-term outcomes. In one study, 91% of patients were still living after 10 years, and 70% were still living after 20 years without needing liver transplants^[16].

Most patients don’t need liver transplants. After 10 years of being diagnosed and treated, only about 9% to 10% of patients needed a liver transplant or died from liver-related disease^[16]. Patients with early-stage disease whose liver enzymes return to normal with treatment have the best prognosis and will likely live long lives without needing a transplant^[16].

An encouraging finding is that liver scarring may be reversible in some patients whose inflammation is well controlled with treatment^[16]. Studies have found that over half of treated patients with follow-up liver biopsies showed some improvement or reduction in liver fibrosis (scarring). This improvement may even be possible for those who have cirrhosis at diagnosis.

However, without treatment, the outlook is very different. Untreated autoimmune hepatitis typically gets worse over time. Only about 12% of untreated patients see the disease resolve on its own^[11]. Up to 70% of untreated patients will develop symptoms or cirrhosis within 10 years, and untreated disease significantly reduces life expectancy^[11].

The key to a good outcome is early diagnosis and consistent treatment. With proper medical care, regular monitoring, and adherence to medication regimens, most people with autoimmune hepatitis can control their disease, prevent serious complications, and enjoy good quality of life.

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