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Mariusz Klin, M.D., Ph.D

Autoimmune Hepatitus

Autoimmune hepatitis was first described in 1950 as a disease of young women and is associated with increased gamma globulin in the blood and chronic hepatitis in the liver biopsy. The presence of antinuclear antibodies (ANA) and the resemblance of some symptoms to " systemic lupus erythematosus " (SLE) led to the label " lupoid hepatitis " . It later became evident that this disease was not related to SLE. The disease is now called autoimmune hepatitis.

What are the Symptoms?

The typical patient with autoimmune hepatitis is female (70%). The disease may start at any age, but it is most common in adolescents or those in early adulthood. Blood tests identify ANA or smooth muscle antibodies (SMA) in the majority of patients (60%). More than 80% of affected individuals have increased gamma globulin in the blood. Some patients have other autoimmune disorders such as thyroiditis, ulcerative colitis, diabetes mellitus, vitiligo (patchy loss of skin pigmentation), or Sjogren's syndrome (a syndrome that causes dry eyes and dry mouth). Other liver diseases such as viral hepatitis, Wilson's disease, hemochromatosis, and alpha-1-antitrypsin deficiency should be excluded by appropriate blood tests, and the possibility of drug-induced hepatitis is ruled out by careful questioning.

The most common symptoms of autoimmune hepatitis are fatigue, abdominal discomfort, aching joints, itching, jaundice, enlarged liver, and spider angiomas (blood vessels) on the skin. Patients may also have complications of more advanced chronic hepatitis with cirrhosis, such as ascites (abdominal fluid) or mental confusion called encephalopathy. A liver biopsy is important to confirm the diagnosis and provide a prognosis. A liver biopsy may show mild autoimmune hepatitis, more advanced chronic hepatitis with scarring (fibrosis), or fully developed cirrhosis.

How is Autoimmune Hepatitis Treated?

The ten-year survival rate in untreated patients is approximately 10%. The treatment of autoimmune hepatitis is immunosuppression. This medical therapy has been shown to decrease symptoms, improve liver tests, and prolong survival in the majority of patients.

The goal of treatment for autoimmune hepatitis is to cure, or control, the disease. In two-thirds to three-quarters of the patients, liver tests fall to within the normal range. Long-term follow up studies show that autoimmune hepatitis appears more often to be a controllable rather than a curable disease, because the majority of patients relapse within six months after therapy is ended. Therefore, most patients need long-term maintenance therapy for this condition.

Not all patients with autoimmune hepatitis respond to prednisone treatment. Approximately 15-20% of patients with severe disease continue to deteriorate despite the initiation of appropriate therapy. This is most common in patients with advanced cirrhosis on the initial liver biopsy. Such patients are unlikely to respond to further medical therapy, and liver transplantation should be considered.

Source: AMA Complete Encyclopedia, Copyright 2003, American Medical Association
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