Autoimmune Hepatitis
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 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.
What are the Symptoms?
The typical patient with autoimmune hepatitis is female
(70%). 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%). 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. 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 immunosuppresion. This medical therapy has been shown
to decrease symptoms, improve liver tests, and prolong
survival in the majority of patients.
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% 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.
Source: AMA Complete Medical Encyclopedia,
Copyright 2003, American Medical Association
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