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

Lupoid hepatitis; Chronic acute liver disease

This form of hepatitis is an autoimmune disease. The body's 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. This suggests that there may be a genetic cause.

This disease is most common in young girls and women.

• 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).

Tests for autoimmune hepatitis include:

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

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

Some people may need a liver transplant.

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

• Cirrhosis
• Side effects from steroids and other medicines
• Hepatocellular carcinoma
• Liver failure

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

Autoimmune hepatitis is usually cannot be prevented. Knowing the risk factors may allow early detection and treatment.

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.