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

This disease is associated with other autoimmune diseases, including:

• Hemolytic anemia
• Proliferative glomerulonephritis
• 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.

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

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

• Abnormal liver function tests
• Liver biopsy showing chronic hepatitis

Tests associated with autoimmune hepatitis:

• ANA
• Anti-liver kidney microsomal antibody
• Anti-mitochondrial antibody
• Sedimentation rate
• Serum IgG

Prednisone or other corticosteroid medications help reduce the inflammation. Azathioprine and 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.

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

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

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

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

Lupoid hepatitis

Luxon BA. Diagnosis and treatment of autoimmune hepatitis. Gastroenterology Clinics. June 2008;37.

Updated by: George F. Longstreth, MD, Department of Gastroenterology, Kaiser Permanente Medical Care Program, San Diego, CA. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.