<< Back to Infosheets

Autoimmune Hepatitis

What is Autoimmune Hepatitis?

Autoimmune hepatitis is a disease in which the body's immune system attacks liver cells. First described in 1950 as a disease of young women, autoimmune hepatitis is associated with increased gamma globulin and antibodies in the blood, progressive inflammation of the liver due to immune system abnormality and liver biopsy indicating chronic hepatitis. This disease has been identified by a number of different names (lupoid hepatitis, autoimmune chronic active hepatitis, idiopathic chronic active hepatitis). Although not related, it has some symptoms similar to systemic lupus erythematosus and the presence of antinuclear antibodies. About 70 percent of those with autoimmune hepatitis are female.

What Are the Symptoms?

A liver biopsy is important to confirm diagnosis and provide prognosis. A biopsy may show mild chronic active hepatitis, advance chronic active hepatitis with some scarring (fibrosis), or fully advanced cirrhosis. The most common symptoms of autoimmune hepatitis are enlarged liver, fatigue, aching joints, abdominal discomfort, jaundice and spider angiomas (tumors) on the skin. With advanced disease, patients may have complications of cirrhosis including ascites or retention of fluid in the abdomen and mental confusion known as encephalopathy.

Autoimmune hepatitis may start at any age, but is most common (70%) among adolescent or young adult females. In the majority of patients (60%), blood tests show smooth muscle antibodies-SMA. More than 80% of patients have high gamma globulin blood levels. Some individuals may also have other autoimmune disorders such as diabetes mellitus, vitiligo (lost of skin pigmentation), ulcerative colitis, thyroiditis, or Sjogren's syndrome (dry eyes and mouth). Appropriate testing and evaluation will rule out other liver diseases such as viral hepatitis, hemochromatosis, alpha 1-antitypsin deficiency, Wilson's disease and drug-induce hepatitis.

Is There Treatment for Autoimmune Hepatitis?

Autoimmune hepatitis is treated with immunosuppressant drugs such as prednisone alone or prednisone and azathioprine (Imuran). This treatment has been shown to improve liver tests, decrease symptoms and prolong survival in most patients. Treatment is usually started with 30 to 40 mg of prednisone per day. The dosage is reduced after the patient has responded to treatment. The standard dosage for the majority of patients is 10-15 mg of prednisone per day, alone or in combination with 50 mg of azathioprine per day. Lower doses of prednisone may be administered when combined with azathioprine. Long-term high doses of prednisone are associated with serious side effects: hypertension, diabetes, peptic ulcer, bone thinning, cataracts.

People who do not respond to standard immune therapy or who have severe side effects may benefit from other immunosuppressive agents such as mycophenylate mofetil, cyclosporine, or tacrolimus.

Although the goal of the treatment is to cure or control the disease, follow-up studies have demonstrated that autoimmune hepatitis is more controllable than curable. Due to the fact that the majority of patients relapse after six months, most patients need long-term maintenance therapy. Liver function tests fall to within normal range in 2/3-3/4 of the patients. In untreated patients the ten-year survival rate is approximately 10%.

An estimated 15-20% of the patients with advanced cirrhosis on initial biopsy continue to deteriorate despite undergoing appropriate therapy. These patients are not likely to respond to further therapy and in this case, a liver transplant may be considered.

© Copyright 2000 - The Latino Organization for Liver Awareness