What Is Autoimmune Hepatitis

The immune system is the body’s defence mechanism against harmful foreign substances such as bacteria and viruses. However, in some cases, the immune system may mistake the body’s own cells for a foreign entity and attack them. This is what causes immune hepatitis. Immune hepatitis is a type of chronic liver disease, which is caused by the body’s immune system mistaking healthy liver cells for a foreign substance. The immune system’s attack on the liver cells leads to inflammation and damage of the liver.¹ The symptoms of autoimmune hepatitis are similar to those of other hepatitis types, and therefore a correct diagnosis is vital to ensure you receive the correct treatment.

Overview

Autoimmune hepatitis is the result of your body’s own immune system attacking the cells of your liver. This continuous attack causes the liver to become inflamed and damaged. Currently, there is no exact cause known for this chronic and progressive disease.¹ This article will help you identify the risk factors and symptoms associated with autoimmune hepatitis, learn how it is diagnosed, and educate you on the treatments currently available for this autoimmune disease.

Types of autoimmune hepatitis

There are two clinical types of autoimmune hepatitis identified by experts, these are type 1 and type 2.²

Type 1 autoimmune hepatitis accounts for 80% of autoimmune hepatitis diagnosis. While it can develop at any age,it is most commonly diagnosed between the ages of 15 and 40, particularly in people assigned female at birth (AFAB). This form of autoimmune hepatitis is considered less severe and is often associated with autoimmune disorders such as coeliac disease, thyroiditis, and ulcerative colitis.² 

Type 2 autoimmune hepatitis accounts for 10-20% of autoimmune hepatitis cases. This type is usually diagnosed in children and young adults. It is characterised by the presence of specific anti-liver and anti-kidney antibodies. This type of autoimmune hepatitis can be more severe and, as such, more difficult to treat. It is usually associated with other autoimmune disorders including autoimmune thyroid disease and type 1 diabetes.²

Causes of autoimmune hepatitis

Currently, the exact cause of autoimmune hepatitis is unknown, however, it is assumed to be the result of a combination of immune, genetic and environmental factors.³

There is a hereditary element to autoimmune hepatitis shown through its tendency to run in families. Investigations have shown that specific genetic variations correlate with an increased risk of autoimmune hepatitis development.^4,5

In those with a genetic predisposition to this autoimmune disease, environmental factors can contribute to the triggering of the disease. These environmental factors can include infection with the Epstein-Barr, herpes simplex or measles viruses, certain medications, and toxin exposure.^{6, 7}

As stated above, autoimmune hepatitis arises from the attack of liver cells by the body’s immune system. Whilst the cause of this is not clear, it is apparent that immune dysregulation plays a key role in the development of autoimmune hepatitis. Furthermore, autoimmune hepatitis is frequently associated with other autoimmune disorders including coeliac disease, thyroiditis and ulcerative colitis. This suggests the same immune dysregulations could be responsible for the development of different types of inflammatory autoimmune diseases.

Signs and symptoms of autoimmune hepatitis

The signs and symptoms of autoimmune hepatitis vary between individuals and develop at different rates. The most common signs and symptoms of the disease listed on NORD include:

• Fatigue and weakness
• Jaundice (skin and white of the eyes appear yellow)
• Spider-like Rashes
• Pain or discomfort in the abdomen
• Loss of menstruation in people AFAB
• Liver enlargement
• Joint pain and stiffness

You should see a doctor if you experience any of these symptoms. 

Management and treatment for autoimmune hepatitis

Autoimmune hepatitis can be managed and treated using a combination of medicines and lifestyle changes. As there is no cure for autoimmune hepatitis, treatments aim to reduce inflammation, therefore preventing additional liver damage and thus providing an overall improved quality of life for patients. Generally speaking, the prognosis for patients with autoimmune hepatitis is good and most patients will achieve remission. However, lifelong treatment and monitoring may be necessary.^{3, 8}

Immunosuppressants are the main medications used for the treatment of autoimmune hepatitis. They work to reduce and prevent the immune system from attacking liver cells, thus helping control inflammation. Examples of immunosuppressants that may be used include prednisone and azathioprine. Other medications may also be used to improve liver function.⁸

For those with autoimmune hepatitis, it is very important to maintain a healthy lifestyle and avoid alcohol consumption, so as to prevent further liver damage. It is also important to note that individuals with autoimmune hepatitis should consult with a doctor before taking new medications, and avoid medications that can cause liver damage including nonsteroidal anti-inflammatory drugs.⁸

In order to ensure optimum treatment for autoimmune hepatitis, a patient’s condition will be monitored closely.

In severe cases, a liver transplant might be necessary.

Diagnosis

Autoimmune hepatitis is diagnosed through a combination of blood tests, imaging tests and a liver biopsy. A doctor may also ask about the patient’s medical history.⁸

Blood tests are used to assess liver function and the presence of antibodies associated with autoimmune hepatitis.

Imaging tests such as a CT scan, ultrasound or MRI are used to visualise the liver and rule out other liver conditions including liver cancer and cirrhosis.

A liver biopsy involves taking a small sample of tissue from the liver. This is then examined under a microscope and assessed for scarring, damage and inflammation. 

Early diagnosis and treatment prevent further liver damage and improve the outcome for the patient.

Risk factors

The exact cause of autoimmune hepatitis is unknown, however, risk factors that increase the likelihood of developing the disease include:^{9, 4, 5, 6, 7}

• Gender: People AFAB are more likely to develop autoimmune hepatitis
• Age: People between 15-40 years old are more commonly diagnosed with autoimmune hepatitis
• Family history of autoimmune diseases
• Infection with measles, herpes simplex or Epstein-Barr viruses
• Having other autoimmune diseases
• Medications including minocycline, nitrofurantoin and statins

It should be noted that having one or more of these risk factors doesn’t necessarily mean that you will develop autoimmune hepatitis.

Complications

Autoimmune hepatitis complications can occur if the condition is left untreated or poorly managed. In severe cases, these can include cirrhosis, liver failure and liver cancer.

In addition, some medications used to treat autoimmune hepatitis can increase the risk of osteoporosis and increase the likelihood of infections.

FAQs

How can I prevent autoimmune hepatitis

As the exact cause of the disease is not understood, it is not possible to prevent autoimmune hepatitis.

How common is autoimmune hepatitis

Autoimmune hepatitis is an uncommon disease. According to the British Liver Trust, in Europe, it affects between 10 and 17 people per 100,000. It is 3 to 4 times more likely to develop in people AFAB.

Can autoimmune hepatitis be cured

Autoimmune hepatitis cannot be cured, but it can be effectively managed with appropriate treatment. With correct treatment, autoimmune hepatitis can go into remission. This means the liver function returns to normal and is maintained as such. However, ongoing monitoring and treatment will still be necessary to prevent a relapse and manage the disease.

When should I see a doctor

You should see your doctor if you have any of the signs or symptoms of autoimmune hepatitis. Furthermore, if you have a family history of autoimmune diseases, you may be at an increased risk of developing autoimmune hepatitis and should talk to your doctor about your individual risk factors.

If you have already been diagnosed with autoimmune hepatitis, you should see your doctor regularly to manage the disease.

Summary

To summarise, autoimmune hepatitis is the result of the body’s immune system attacking liver cells. This attack causes the liver to become inflamed and damaged. Currently, there is no cure for this disease. However, with treatment, patients with autoimmune hepatitis generally have good prognosis.