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PO Box 267 Southport PR8 1WD
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Primary Sclerosing
Cholangitis Trust
Dedicated to finding a cure for PSC
About PSC

the liver & treatment
URSO & Questran
related conditions
ulcerative colitis
low fat
clear liquids
crohn's disease
fibre restricted
anal fissure, abscess & fistula
rectal bleeding
related procedures
liver biopsy
liver transplant
















PSC is chronic progressive liver disease whose cause and cure is unknown, advancing toward cirrhosis and sometimes liver failure.

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. The bile is then unable to drain through the ducts accumulating in the liver causing damage to liver cells. Eventually, so much bile is accumulated, it seeps into the bloodstream. As a result of this long term cell damage the liver develops cirrhosis (hardening or fibrosis).

The exact cause of PSC is unknown. Researchers suggest that there may be a connection to the immune system, but this is yet unproven.

PSC often starts between the ages of late teens and 20s and 45 and 50's, 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 (IBD), especially ulcerative colitis in which the colon becomes inflamed and ulcerated. Researchers again suggest that genetic factors may link PSC and ulcerative colitis.

PSC usually progresses very slowly. Initially there may be no symptoms unless a blood test picks up for instance above normal ranges in the blood of the liver enzyme levels called alkaline phosphatase. When symptoms do develop, they may be intermittent or persistent. Gradually, they may worsen. The symptoms are caused by two things: the bile not draining properly through the bile ducts, and the liver 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 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.

Although the disease is slow in progressing it varies from one person to another, often liver transplantation is the only therapeutic option for patients with end stage liver disease resulting from this disorder, but there is increased risk of stomach cancer and more aggressive IBD including Ulcerative Colitis with no guarantee that the PSC won’t attack the new liver.