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Primary Biliary Cirrhosis
Primary Sclerosing Cholangitis (PSC)
| | Primary Sclerosing Cholangitis (PSC)
The Liver
The liver is the largest organ in the body. It is found high in the right upper
abdomen, behind the ribs. It is a very complex organ and has many functions.
They include:
 | Storing energy in the form of sugar (glucose)
| Storing vitamins, iron, and other minerals
| Making proteins, including blood clotting factors, to keep the body
healthy and help it grow
| Processing worn out red blood cells
| Making bile which is needed for food digestion
| Metabolizing or breaking down many medications and alcohol
| Killing germs that enter the body through the intestine |
| | | | | |
The liver cells excrete bile into tiny tubes within the liver called bile
ducts. These tubes come together like the tiny veins on a leaf. They drain the
bile into the common bile duct, a larger single tube leading into the intestine.
There the bile aids digestion and gives stool its brown color. As you can see,
the liver is a very important organ. 
What is Primary Sclerosing Cholangitis (PSC)?
Primary sclerosing cholangitis is a disease primarily of the bile ducts, both
inside and outside the liver. The ducts of the gallbladder and pancreas may also
be involved. The walls of the bile ducts become inflamed (cholangitis). The
inflammation causes scarring and hardening (fibrosis) that narrows the bile
ducts. Because bile cannot drain properly through the ducts, it accumulates in
the liver causing damage to liver cells. Eventually, so much bile is
accumulated, it seeps into the bloodstream. Finally, with long term cell damage,
the liver develops cirrhosis (hardening or fibrosis) and it can no longer
function properly.
Cause
The exact cause of PSC is unknown. However, the most likely cause appears to be
changes in the way the immune system works. When the immune system is working
properly, it protects the body from infections caused by foreign invaders like
bacteria and viruses. Sometimes, however, it recognizes certain body parts or
organs as foreign. The body then goes to war against itself, damaging the body
part it thinks is foreign.
PSC often starts between the ages of 30 and 50, and it occurs most often in
men. It was once considered a rare disease, but recent studies show it is more
common than previously thought. About 70% of the patients with PSC also have an
inflammatory bowel disease, especially ulcerative colitis in which the colon
becomes inflamed and ulcerated. Medical experts believe genetic factors may link
PSC and ulcerative colitis.
Symptoms
PSC usually progresses very slowly. Early on there may be no symptoms. Usually
the only findings are abnormal laboratory test results. For example, a liver
enzyme called alkaline phosphatase may be above normal ranges in the blood. When
symptoms do develop, they may be intermittent or persistent. Gradually, they may
worsen. The symptoms are caused by two things: the bile is not being drained
properly through the bile ducts, and the liver is not doing its job. Bile ducts
can become infected, causing chills, fever and upper abdominal tenderness.
Itching may occur when bile seeps into the bloodstream. As the disease
progresses, chronic fatigue, loss of appetite, weight loss and jaundice
(yellowing of skin and eyes) may occur. Finally, in the advanced stages of
cirrhosis, extensive swelling can occur in the abdomen and feet. Liver failure
may take many years to develop.
 Diagnosis
The physician may suspect PSC from the patient's medical history, especially a
history of inflammatory bowel disease, and from abnormal blood tests. The
diagnosis is usually made by cholangiography, an x-ray called ERCP that involves
injecting dye into the bile ducts. The test is performed under sedation. A
lighted, flexible endoscope is inserted through the mouth, stomach and then into
the small intestine. A thin tube is place through the scope into the bile ducts,
and the dye is injected to highlight the bile ducts on the x-ray. If there is
narrowing of the bile ducts, the diagnosis of PSC is confirmed.
As the disease progresses, a liver biopsy is usually needed to determine how
much damage has occurred. Under local anesthesia, a slender needle is inserted
through the right lower chest to extract a small piece of liver for microscopic
analysis. 
Treatment
At the present time there is no cure for PSC, but effective treatment is
available. There are a number of ways to treat symptoms and the various stages
of the disease. Itching, from too much bile in the bloodstream, can be
controlled with drugs such as Questran. Bile is usually reabsorbed into the
bloodstream from the large intestine, and goes back to the liver to be reused.
Questran binds up bile in the intestine, allowing it to be eliminated with the
stool instead. This helps to reduce the build-up of bile in the body. Actigall
is a drug that favorably changes the make-up of bile in the liver. This, in
turn, seems to reduce the amount of liver damage that occurs. Results of early
medical studies seem to indicate that this drug may increase survival. Sometimes
the bile ducts become infected and must be treated with antibiotics. If
ulcerative colitis is also present, it is treated with the appropriate
medicines. Swelling of the abdomen and feet, due to fluid retention from
cirrhosis, can be treated with a salt-restricted diet and diuretics (fluid
pills). Presently, there are exciting studies being done to test the
effectiveness of other drugs on the body's immune system, since this seems to be
the underlying problem.
In some cases, endoscopic or surgical procedures may be used to open major
blockages in bile ducts. Through an endoscope, the physician places a tiny tube
with a balloon on the end into the narrowed bile duct. The balloon is inflated
to expand the duct so bile can flow through it once again. Sometimes stents
(plastic tubing) can be placed in the narrowed ducts to keep them open. Often
PSC progresses to a point where liver transplantation must be considered.
Liver Transplantation
Liver transplantation is now an accepted form of treatment for chronic, severe
liver disease. Advances in surgical techniques and the use of new drugs to
suppress rejection have improved the success rate of transplantation. The
outcome for PSC patients is excellent. Because of the disease's slow progress,
it is possible to plan elective transplant surgery. Survival rates at transplant
centers are well over 90 percent, with a good quality of life after recovery.
Summary
Primary sclerosing cholangitis is a slow, progressive disease. Once diagnosed,
treatment is directed at managing symptoms and opening narrowed bile ducts. A
great deal of research is underway aimed at preventing damage to the bile ducts,
improving symptoms and prolonging life. By working closely with the physician,
there is good reason to expect a favorable long-term outlook.
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