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 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.

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

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.

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.

Complications may include:

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

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

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

Lupoid hepatitis; Chronic active hepatitis

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.

Updated by: Michael M. Phillips, MD, Emeritus Professor of Medicine, The George Washington University School of Medicine, Washington, DC. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.