Autoimmune hepatitis

Alternative names
Lupoid hepatitis

Definition
Autoimmune hepatitis involves inflammation of the liver caused by rogue immune cells that mistake the liver’s normal cells for a foreign tissue or pathogen (disease-causing agent).

Causes, incidence, and risk factors

A person with autoimmune hepatitis has autoantibodies circulating in the bloodstream that cause the immune system to attack the liver.

This disease is associated with other autoimmune diseases, including the following:

     
  • Thyroiditis  
  • Type 1 diabetes  
  • Ulcerative colitis  
  • Hemolytic anemia  
  • Proliferative glomerulonephritis

Autoimmune hepatitis sometimes occurs in relatives of people with autoimmune diseases, suggesting a genetic cause.

This disease is most common in young girls and women.

Symptoms

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

Additional symptoms that may be associated with this disease include amenorrhea (absence of menstruation).

Signs and tests

     
  • Liver biopsy showing chronic hepatitis  
  • Abnormal liver function tests

Tests associated with autoimmune hepatitis:

     
  • Positive ANA  
  • Positive anti-smooth muscle antibody  
  • Positive anti-liver kidney microsomal antibody  
  • Positive anti-mitochondrial antibody  
  • Elevated sedimentation rate  
  • Elevated serum IgG

Treatment

Prednisone or other corticosteroids help reduce the inflammation. Azathioprine or mercaptopurine are drugs used to treat other autoimmune disorders, and they have benefitted patients with autoimmune hepatitis as well.

Modify your activity level according to your symptoms.

Expectations (prognosis)

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

Complications

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

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.

Johns Hopkins patient information

Last revised: December 4, 2012
by Janet G. Derge, M.D.

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All ArmMed Media material is provided for information only and is neither advice nor a substitute for proper medical care. Consult a qualified healthcare professional who understands your particular history for individual concerns.