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Autoimmune Hepatitis

Overview

Autoimmune hepatitis (AIH) is liver inflammation caused by your immune system attacking liver cells. The exact etiology of autoimmune hepatitis is unknown, but genetic and environmental factors appear to interact over time to cause the disease. Autoimmune hepatitis, if left untreated, can lead to liver scarring (cirrhosis) and eventually liver failure. However, if diagnosed and treated early, autoimmune hepatitis can often be controlled with immune-suppressing drugs. When autoimmune hepatitis does not respond to medication or the liver is severely damaged, a liver transplant may be an option.

Autoimmune hepatitis (AIH) is liver inflammation caused by your immune system attacking liver cells. The exact etiology of autoimmune hepatitis is unknown, but genetic and environmental factors appear to interact over time to cause the disease. Autoimmune hepatitis, if left untreated, can lead to liver scarring (cirrhosis) and eventually liver failure. However, if diagnosed and treated early, autoimmune hepatitis can often be controlled with immune-suppressing drugs. When autoimmune hepatitis does not respond to medication or the liver is severely damaged, a liver transplant may be an option.

Types

The two types of autoimmune hepatitis are: 

Type 1 Autoimmune Hepatitis: This is the most common type of the disease. Anyone of any age can be affected. Approximately half of the people with type 1 autoimmune hepatitis have other autoimmune disorders like rheumatoid arthritisceliac disease, or ulcerative colitis. This is the most common type of Autoimmune Hepatitis in the US, accounting for 96% of all AIH cases in North America. It has a 4 to 1 female to male ratio and a good response to corticosteroids.

Type 2 Autoimmune Hepatitis: Patients with this type of disease are typically younger (less than 14 years old), have more severe disease, have a poorer response to corticosteroids, and relapse more often. Although adults can get type 2 autoimmune hepatitis, it is most frequent in children and teenagers.  In North America, Type 2 AIH accounts for only 4% of all AIH cases. Other autoimmune disorders may accompany this type of autoimmune hepatitis.

The two types of autoimmune hepatitis are: 

Type 1 Autoimmune Hepatitis: This is the most common type of the disease. Anyone of any age can be affected. Approximately half of the people with type 1 autoimmune hepatitis have other autoimmune disorders like rheumatoid arthritisceliac disease, or ulcerative colitis. This is the most common type of Autoimmune Hepatitis in the US, accounting for 96% of all AIH cases in North America. It has a 4 to 1 female to male ratio and a good response to corticosteroids.

Type 2 Autoimmune Hepatitis: Patients with this type of disease are typically younger (less than 14 years old), have more severe disease, have a poorer response to corticosteroids, and relapse more often. Although adults can get type 2 autoimmune hepatitis, it is most frequent in children and teenagers.  In North America, Type 2 AIH accounts for only 4% of all AIH cases. Other autoimmune disorders may accompany this type of autoimmune hepatitis.

Causes

Autoimmune hepatitis develops when the body's immune system, which normally attacks viruses, bacteria, and other pathogens, attacks the liver instead. This attack on your liver may cause chronic inflammation and damage your liver cells. Although the exact etiology of autoimmune hepatitis is unknown, experts believe it is caused by the interaction of genes that control immune system function. Environmental factors may induce an autoimmune reaction in people with these genes, causing their immune system to attack their liver. Environmental causes that have a role in autoimmune hepatitis are currently being researched. Viruses and medications are examples of possible triggers.

Autoimmune hepatitis develops when the body's immune system, which normally attacks viruses, bacteria, and other pathogens, attacks the liver instead. This attack on your liver may cause chronic inflammation and damage your liver cells. Although the exact etiology of autoimmune hepatitis is unknown, experts believe it is caused by the interaction of genes that control immune system function. Environmental factors may induce an autoimmune reaction in people with these genes, causing their immune system to attack their liver. Environmental causes that have a role in autoimmune hepatitis are currently being researched. Viruses and medications are examples of possible triggers.

Signs And Symptoms

Autoimmune hepatitis signs and symptoms differ from person to person and might appear suddenly. They may include: 

Epidemiology

Autoimmune hepatitis is a condition that affects people worldwide, although the precise incidence and prevalence in the United States are unknown. The annual prevalence and incidence of AIH in Northern Europeans are about 18 per 100,000 people and 1.1 per 100,000 people, respectively. These statistics can be generalized to the North American population. AIH is substantially more common in Native Alaskans, with a point prevalence of 42 per 100,000 people per year. Females are more likely to develop autoimmune hepatitis, with a bimodal age distribution with two peaks, one in childhood and the other in the fifth decade. However, AIH affects both men and women of all ages, and there have been reports of newly diagnosed AIH in people as old as 80.

Autoimmune hepatitis is a condition that affects people worldwide, although the precise incidence and prevalence in the United States are unknown. The annual prevalence and incidence of AIH in Northern Europeans are about 18 per 100,000 people and 1.1 per 100,000 people, respectively. These statistics can be generalized to the North American population. AIH is substantially more common in Native Alaskans, with a point prevalence of 42 per 100,000 people per year. Females are more likely to develop autoimmune hepatitis, with a bimodal age distribution with two peaks, one in childhood and the other in the fifth decade. However, AIH affects both men and women of all ages, and there have been reports of newly diagnosed AIH in people as old as 80.

Risk Factors

According to studies, several factors can raise your risk of getting autoimmune hepatitis, including:

Being a woman: Autoimmune hepatitis can affect both men and women; however, females are more likely to get the disease.

Infections in the past: You may develop autoimmune hepatitis after contracting the measles, herpes simplex, or Epstein-Barr virus. Infection with hepatitis A, B, or C is also linked to the condition.

Having an autoimmune disease: People with celiac disease, rheumatoid arthritis, hyperthyroidism (Hashimoto’s thyroiditis or Graves' disease), type 1 diabetes, hemolytic anemia, immune thrombocytopenia are more likely to develop autoimmune hepatitis.

Inheritance: A predisposition to autoimmune hepatitis appears to run in families.

According to studies, several factors can raise your risk of getting autoimmune hepatitis, including:

Being a woman: Autoimmune hepatitis can affect both men and women; however, females are more likely to get the disease.

Infections in the past: You may develop autoimmune hepatitis after contracting the measles, herpes simplex, or Epstein-Barr virus. Infection with hepatitis A, B, or C is also linked to the condition.

Having an autoimmune disease: People with celiac disease, rheumatoid arthritis, hyperthyroidism (Hashimoto’s thyroiditis or Graves' disease), type 1 diabetes, hemolytic anemia, immune thrombocytopenia are more likely to develop autoimmune hepatitis.

Inheritance: A predisposition to autoimmune hepatitis appears to run in families.

Diagnosis

The following tests and procedures are used to diagnose autoimmune hepatitis:

Blood Tests: Antibodies can be detected in a blood sample to differentiate autoimmune hepatitis from viral hepatitis and other conditions with similar symptoms. Antibody tests can also be used to determine which type of autoimmune hepatitis you have.  . Antinuclear antibody (ANA) and anti-smooth muscle antibody (ASMA) are present in Type 1 Autoimmune Hepatitis. In Type 2 Autoimmune Hepatitis, anti-liver kidney microsomal antibody type 1 (anti-LKM1) and/or anti-liver cytosol type 1 (anti-LC1) autoantibodies are present.

The following are some of the other lab blood tests used to diagnose autoimmune hepatitis:

Liver function tests:  These tests look for signs of liver inflammation or damage.

CBC (complete blood count):  The number and types of cells in your blood are examined.

Coagulation panel:  This test examines the effectiveness of the clotting proteins.

Electrolytes panel: This test is used to determine whether you have an electrolyte imbalance.

Imaging tests such as:

CT scan:  This is a more detailed X-ray.  It can provide detailed views of the bones, muscles, fat, and organs in a specific part of the body. 

MRI:  This test creates detailed images of your internal organs and structures.  A dye may be injected into your vein. The dye helps visualize the liver and other abdominal organs during the scan.

Ultrasound:  This creates an image of the organs using high-frequency sound waves. 

Liver Biopsy: A liver biopsy is used to confirm the diagnosis and identify the extent and type of liver damage. A thin needle is introduced into your liver through a small incision in your skin, and a small amount of liver tissue is removed during the treatment and then sent to a lab for testing.

The following tests and procedures are used to diagnose autoimmune hepatitis:

Blood Tests: Antibodies can be detected in a blood sample to differentiate autoimmune hepatitis from viral hepatitis and other conditions with similar symptoms. Antibody tests can also be used to determine which type of autoimmune hepatitis you have.  . Antinuclear antibody (ANA) and anti-smooth muscle antibody (ASMA) are present in Type 1 Autoimmune Hepatitis. In Type 2 Autoimmune Hepatitis, anti-liver kidney microsomal antibody type 1 (anti-LKM1) and/or anti-liver cytosol type 1 (anti-LC1) autoantibodies are present.

The following are some of the other lab blood tests used to diagnose autoimmune hepatitis:

Liver function tests:  These tests look for signs of liver inflammation or damage.

CBC (complete blood count):  The number and types of cells in your blood are examined.

Coagulation panel:  This test examines the effectiveness of the clotting proteins.

Electrolytes panel: This test is used to determine whether you have an electrolyte imbalance.

Imaging tests such as:

CT scan:  This is a more detailed X-ray.  It can provide detailed views of the bones, muscles, fat, and organs in a specific part of the body. 

MRI:  This test creates detailed images of your internal organs and structures.  A dye may be injected into your vein. The dye helps visualize the liver and other abdominal organs during the scan.

Ultrasound:  This creates an image of the organs using high-frequency sound waves. 

Liver Biopsy: A liver biopsy is used to confirm the diagnosis and identify the extent and type of liver damage. A thin needle is introduced into your liver through a small incision in your skin, and a small amount of liver tissue is removed during the treatment and then sent to a lab for testing.

Treatment

The goal of treatment, regardless of which type of autoimmune hepatitis you have, is to slow or stop the immune system's attack on your liver. This could help slow the disease's progression.

Medication: You'll need immune system-suppressing drugs to achieve this goal. Prednisone is frequently used as the first line of defense. In addition to prednisone, another medicine called azathioprine (Azasan, Imuran) may be prescribed.  Prednisone can cause various adverse effects when taken for longer, including bone thinning (osteoporosis), broken bones (osteonecrosis), diabetes, cataracts, glaucoma, high blood pressure, and weight gain, especially when used long-term. Prednisone is usually prescribed at a high dose during the first month of treatment. Then, the dose is gradually reduced over several months until the disease is controlled at the lowest possible dose to limit adverse effects. You can also avoid prednisone side effects by taking azathioprine with it.  Even if you go into remission a few years after initiating treatment, the disease will usually return if the drug is stopped. You may require treatment for the rest of your life, depending on the situation.

Liver Transplant: When medications fail to stop the disease from progressing, or you develop irreversible scarring (cirrhosis) or liver failure, the only option left is a liver transplant. Your damaged liver is removed and replaced by a healthy liver from a donor during a liver transplant.

The goal of treatment, regardless of which type of autoimmune hepatitis you have, is to slow or stop the immune system's attack on your liver. This could help slow the disease's progression.

Medication: You'll need immune system-suppressing drugs to achieve this goal. Prednisone is frequently used as the first line of defense. In addition to prednisone, another medicine called azathioprine (Azasan, Imuran) may be prescribed.  Prednisone can cause various adverse effects when taken for longer, including bone thinning (osteoporosis), broken bones (osteonecrosis), diabetes, cataracts, glaucoma, high blood pressure, and weight gain, especially when used long-term. Prednisone is usually prescribed at a high dose during the first month of treatment. Then, the dose is gradually reduced over several months until the disease is controlled at the lowest possible dose to limit adverse effects. You can also avoid prednisone side effects by taking azathioprine with it.  Even if you go into remission a few years after initiating treatment, the disease will usually return if the drug is stopped. You may require treatment for the rest of your life, depending on the situation.

Liver Transplant: When medications fail to stop the disease from progressing, or you develop irreversible scarring (cirrhosis) or liver failure, the only option left is a liver transplant. Your damaged liver is removed and replaced by a healthy liver from a donor during a liver transplant.

Complications

Untreated autoimmune hepatitis can lead to permanent scarring of the liver tissue (cirrhosis). Cirrhosis can cause the following complications:

  • Abdominal ascites ( Fluid accumulation)
  • Esophageal varices ( enlarged veins in the esophagus)
  • Mental Confusion
  • Internal bleeding
  • Liver cancer
  • Liver failure

Lifestyle Modifications

Changes in your lifestyle can help you maintain your health. Avoid drinking and eat a well-balanced diet with plenty of fruits and vegetables. Do not take any medications or supplements without first consulting your doctor.

Changes in your lifestyle can help you maintain your health. Avoid drinking and eat a well-balanced diet with plenty of fruits and vegetables. Do not take any medications or supplements without first consulting your doctor.

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