Chronic autoimmune hepatitis is a condition which predominantly affects females. It has an 8:1 female to male predominance.

Autoimmune hepatitis can be subcategorized into six primary subtypes. Type 1, also known as lupoid, is the most common. In these patients, an ANA antibody, which is a serum test, and an SMA, which is another serum test, will be positive. Type 2a predominantly has only antibodies in the serum against liver kidney microsomes, also known as LKM. Type 2b is very uncommon in the United States and is found predominantly in Europe in patients who have hepatitis C. In these patients with chronic hepatitis C infection in Europe, approximately 7% of these will have LKM antibody present in their serum. Interestingly, these patients with hepatitis C and the presence of LKM respond better to antiviral therapy against their hepatitis C versus therapy directed against the autoimmune portion of their hepatic disease. Type 3 is associated with a positive ANA and SMA. Lastly, two conditions known as autoimmune cholangiopathy and primary biliary cirrhosis will have a primarily ANA and AMA respectively positive.

Chronic autoimmune hepatitis, the most common being type 1, again known as lupoid hepatitis, was first described in 1950 by Waldenstrom. This is a disease that primarily affects young people, primarily women. The exact etiology is unknown. There appear to be defects in suppressor T-cells. This results in the production of autoantibodies against hepatocyte surface antigens. It is not known whether this is an acquired or primary dysregulation of the immune system.

The injury in type 1 autoimmune hepatitis can vary from severe chronic hepatitis to near normal activity of the liver. Under the microscope, large lymphocytes and plasma cells are noted to be infiltrating the liver cells. This condition tends to clinically present in young women, but there seems to be a bimodal presentation in that the next group of patients mostly diagnosed are in their fourth and sixth decades of life. The clinical symptoms tend to be insidious with general malaise, which may progress to outright jaundice, or yellowing of the skin and eyes. Classic treatment is with the initiation of steroids. This may take up to two years of treatment. Confirmation of a “cure” is continuous assessment of liver function studies while on a course of prednisone, along with a liver biopsy to confirm resolution of the hepatitis. Many patients, though, will not respond to prednisone alone and they require other medications, specifically, azathioprine.

For more information on autoimmune hepatitis, please consult your Borland-Groover Clinic physician.

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