Definition

Autoimmune hepatitis causes inflammation of the liver. It occurs when immune cells mistake the liver's normal cells for harmful invaders and attack them.

Alternative Names

Lupoid hepatitis; Chronic active hepatitis

Causes

This form of hepatitis is an autoimmune disease. The body's immune system cannot tell the difference between healthy body tissue and harmful, outside substances. The result is an immune response that destroys normal body tissues.

Liver inflammation, or hepatitis, may occur along with other autoimmune diseases. These include:

• Graves disease
• Inflammatory bowel disease
• Rheumatoid arthritis
• Scleroderma
• Sjögren syndrome
• Systemic lupus erythematosus
• Thyroiditis
• Type 1 diabetes
• Ulcerative colitis

Autoimmune hepatitis may occur in family members of people with autoimmune diseases. There may be a genetic cause.

This disease is most common in young girls and women.

Symptoms

Symptoms may include:

• Fatigue
• General discomfort, uneasiness, or ill feeling (malaise)
• Itching
• Loss of appetite
• Nausea or vomiting
• Joint pain
• Pale or clay-colored stools
• Dark urine
• Abdominal distention

Absence of menstruation (amenorrhea) may also be a symptom.

Exams and Tests

Tests for autoimmune hepatitis include the following blood tests:

• Liver function tests
• Anti-liver kidney microsome type 1 antibody (anti LKM-1)
• Anti-nuclear antibody (ANA)
• Anti-smooth muscle antibody (SMA)
• Serum IgG
• Liver biopsy to look for long-term hepatitis

Treatment

You may need prednisone or other corticosteroid medicines to help reduce the inflammation. Azathioprine and 6-mercaptopurine are drugs used to treat other autoimmune disorders. They have also been shown to help people with autoimmune hepatitis.

Some people may need a liver transplant.

Outlook (Prognosis)

The outcome varies. Corticosteroid medicines may slow the progress of the disease. However, autoimmune hepatitis may advance to cirrhosis. This could require a liver transplant.

Possible Complications

Complications may include:

• Cirrhosis
• Side effects from steroids and other medicines
• Hepatocellular carcinoma
• Liver failure
• Bleeding from varices
• Ascites
• Hepatic encephalopathy

When to Contact a Medical Professional

Contact your health care provider if you notice symptoms of autoimmune hepatitis.

Prevention

In most cases, autoimmune hepatitis cannot be prevented. Knowing the risk factors may help you detect and treat the disease early.

References

Czaja AJ. Autoimmune hepatitis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 11th ed. Philadelphia, PA: Elsevier; 2021:chap 90.

Pawlotsky J-M. Chronic viral and autoimmune hepatitis. In: Goldman L, Schafer AI, eds. Goldman-Cecil Medicine. 26th ed. Philadelphia, PA: Elsevier; 2020:chap 140.