Definition

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

Causes, incidence, and risk factors

In patients who have an autoimmune disease, the immune system can't 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, including:

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

Autoimmune hepatitis sometimes occurs in relatives of people with autoimmune diseases, which suggests that there is a genetic cause.

This disease is most common in young girls and women.

Symptoms

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

Other symptoms that may occur with this disease include absence of menstruation (amenorrhea).

Signs and tests

• Abnormal liver function tests
• Liver biopsy showing chronic hepatitis

Tests for autoimmune hepatitis:

• Anti-liver kidney microsome type 1 antibody (anti LKM-1)
• Anti-nuclear antibody (ANA)
• Anti-smooth muscle antibody (SMA)
• Serum IgG

Treatment

Prednisone or other corticosteroid medications help reduce the inflammation. Azathioprine and 6-mercaptopurine are drugs used to treat other autoimmune disorders. They have been shown to help patients with autoimmune hepatitis, as well.

Some patients may receive a liver transplant.

Expectations (prognosis)

The outcome varies. Corticosteroid therapy may slow the disease progression. However, autoimmune hepatitis may worsen to cirrhosis and require a liver transplant.

Complications

• Cirrhosis
• Complications related to steroids and other medications
• Hepatocellular carcinoma
• Liver failure

Calling your health care provider

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

Prevention

Autoimmune hepatitis is usually not preventable. Awareness of risk factors may allow early detection and treatment.

References

Czaia AJ. Autoimmune hepatitis. In: Feldman M, Friedman LS, Brandt LJ, eds. Sleisenger and Fordtran's Gastrointestinal and Liver Disease. 9th ed. Philadelphia, Pa: Saunders Elsevier; 2010:chap 88.