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Autoimmune hepatitis or autoimmune chronic hepatitis is a progressive inflammation of the liver that has been identified by a number of different names, including autoimmune chronic active hepatitis (CAH), idiopathic chronic active hepatitis, and lupoid hepatitis. The reason for this inflammation is not certain, but it is associated with an abnormality of the body's immune system and is often related to the production of antibodies that can be detected by blood tests. Autoimmune
hepatitis was first described in 1950 as a disease of young women, associated
with increased gamma globulin in the blood and chronic hepatitis on
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. The typical patient with autoimmune hepatitis is female (70 percent). The disease may start at any age, but is most common in adolescence or early adulthood. Blood tests identify ANA or smooth muscle antibodies (SMA) in the majority of patients (60 percent). More than 80 percent 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 (tumors) 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. Liver biopsy
may show mild chronic active hepatitis, more advanced chronic active
hepatitis with scarring (fibrosis), or a fully developed cirrhosis.
The 10-year survival rate in untreated patients is approximately 10 percent. The treatment of autoimmune hepatitis is immunosuppression with prednisone alone or prednisone and azathioprine (Imuran). This medical therapy has been shown to decrease symptoms, improve liver tests, and prolong survival in the majority of patients. Therapy is usually begun with prednisone 30 to 40 mg per day and then this dosage is reduced after a response is achieved. The standard dosage used in the majority of patients is prednisone 10-15 mg per day, either alone or with azathioprine 50 mg per day. Higher doses of prednisone given long-term are associated with an increase in serious side effects including hypertension, diabetes, peptic ulcer, bone thinning, and cataracts. Lower doses of prednisone may be used when combined with azathioprine. The goal of treatment of 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. Not
all patients with autoimmune hepatitis respond to prednisone treatment.
Approximately 15-20 percent of patients with severe disease continue
to deteriorate despite initiation of appropriate therapy. This is most
common in patients with advanced cirrhosis on initial liver biopsy.
Such patients are unlikely to respond to further medical therapy, and
liver transplantation should be considered.
The American Liver Foundation is the only national voluntary non-profit health organization dedicated to treating, curing, and preventing hepatitis and other liver and gallbladder diseases through research and education.
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