Primary Sclerosing
Cholangitis
Primary sclerosing cholangitis is more common in men
than women. Initially, many individuals have no symptoms
and the disease is detected because of abnormal laboratory
test results, particularly an enzyme test called alkaline
phosphatase. It usually begins in the 30s, 40s, and
50s, and is commonly associated with fatigue, itching,
and jaundice. Episodes of fever and chills from superimposed
infection in the bile ducts occasionally occur and can
be distressing symptoms. The diagnosis of primary sclerosing
cholangitis is made by cholangiography, an X-ray test
involving injection of dye into the bile ducts. This
is usually accomplished by an endoscopic procedure called
ERCP (endoscopic retrograde cholangiopancreatography)
but also may be done radiologically, surgically, or,
more recently, with magnetic resonance imaging (MRI)
scans.
Courses of Disease
The course of the disease is unpredictable for the
patient, but is generally slowly progressive. The patient
may have the disease for many years before symptoms
develop. Symptoms may persist at a stable level, be
intermittent, or progress gradually. Liver failure may
occur after 7-15 years of disease or even longer. Patients
who have the disease on a longstanding basis may develop
a superimposed tumor of the bile ducts called cholangiocarcinoma,
but this occurs in only 0.5 to 1% of patients per year.
Treatment
There is currently no specific treatment for primary
sclerosing cholangitis. Research is under way to determine
the effectiveness of a number of medications. The various
symptoms of primary sclerosing cholangitis often respond
effectively to medications that control itching, antibiotics
when recurrent infections occur, and vitamins to replace
those that are deficient. In some instances, endoscopic,
radiologic, or surgical techniques may be employed to
open major blockages in the common bile duct and improve
bile flow. When progressive liver failure occurs in
spite of these measures, liver transplantation may be
indicated. Liver transplant patients have about an 87%
one-year and 77% three or more year survival rate and
a good quality of life recovery.
Source: AMA Complete Medical Encyclopedia,
Copyright 2003, American Medical Association
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