Diseases

The specific cause of PSC is still unknown and therefore it seems not to be preventable. It is appropriate for patients with inflammatory bowel disease (especially ulcerative colitis) and their health care professional to be aware of the relationship with primary sclerosing cholangitis, in case symptoms suggestive of the disease arise. However, PSC is seen in only 3% of patients with inflammatory bowel disease.

PSC may remain silent for several years before symptoms arise. In patients with inflammatory bowel disease, the health care professional should have a high suspicion of the diagnosis because of the relationship between PSC and ulcerative colitis.Depending upon how far the disease has progressed and the effect on liver function, physical examination may reveal an enlarged liver, tenderness in the right upper quadrant beneath the ribs, and an enlarged spleen. The skin may be jaundiced or yellow and there can be evidence of scratching due to intense skin itching. In cirrhosis with end stage liver disease, there may be bruising of the skin, a swollen abdomen due to ascites or fluid caused by decreased protein production and decreased blood flow through the scarred liver, gastrointestinal bleeding, and mental confusion because of elevated ammonia levels in the bloodstream.Blood tests are helpful in assessing the liver and potential blockages within the bile ducts. These may include a complete blood count, INR/PTT (that measures blood clotting and the ability of the liver to produce clotting factors), liver function studies including AST, and ALT to assess liver inflammation, alkaline phosphatase and bilirubin which measure the degree of bile blockage.Imaging tests may include ultrasound to look at the liver structure and magnetic resonance cholangiogram, an MRI of the abdomen that can assess the bile duct structure of the liver.If the diagnosis based on laboratory tests and imaging is still in doubt, a biopsy may be performed. A gastroenterologist or interventional radiologist will place a long, fine needle through the skin into the liver to obtain a piece of tissue. This is analyzed under a microscope by a pathologist to make the diagnosis.

The cause of PSC is unknown but it may be an autoimmune disease. PSC is more often seen in men in the 20-30 age range. There is an association with inflammatory bowel, disease, most often with ulcerative colitis and less commonly with Crohn's Disease. People with PSC who do not have inflammatory bowel disease, are more likely to be female and older.

In its early stages, PSC is asymptomatic (there are no symptoms). It is only when the person develops cholestasis because of the inability of bile to drain from the liver, do symptoms occur. These early symptoms include itching and fatigue. As the bilirubin levels in the blood become elevated, jaundice or a yellowish tinge to the skin, may occur. Since there may be liver inflammation, the person may complain of pain beneath the ribs in the right upper quadrant of the abdomen.The inability of the bile ducts to adequately drain reduces the flow of bile and there can be sludge formation in the bile ducts leading to the risk of infection. This may cause fever and increased pain.Because PSC is a progressive disease, symptoms may come and go over several months and years after the diagnosis is made.As the disease progresses, cirrhosis may occur leading to decreased liver function and its associated signs and symptoms. These include bleeding from the esophagus and stomach due to varices, ascites (fluid collection in the abdomen) because of poor protein production by the liver, easy bruising because of decreased platelets in the blood as because they are trapped in the enlarged spleen, and confusion due to hepatic encephalopathy because the liver cannot clear waste products of metabolism.

The cause of PSC is unknown but it has an association with inflammatory bowel disease, especially ulcerative colitis. It is thought that there may be an autoimmune component to the disease, where the body's immune system attacks the bile ducts in the liver and causes them to become, inflamed and narrowed.

PSC is a progressive disease, damaging the bile ducts and reducing the bile flow, ultimately leading to portal hypertension, cirrhosis, and liver failure. Some cancers are associated with primary sclerosing cholangitis including gallbladder cancer, hepatocellular cancer (cancer of the liver cells) and cholangiocarinoma (cancer of the bile ducts). The combination of inflammatory bowel disease and PSC increases the risk of developing cancers of the colon and rectum

Aside from liver transplantation, there are no effective treatments for PSC. In the US, studies suggest that life expectancy ranges from 9 to 18 years if the patient does not undergo liver transplantation. Using different population study models, researchers in the Netherlands concluded that life expectancy may be longer than 21 years from the time when the diagnosis is made.The prognosis and life expectance is poorer in older patients, those who have an enlarged liver and spleen, and in patients who are persistently jaundiced with elevated bilirubin levels in their bloodstream.

The treatment of PSC is supportive, monitoring the progression of the disease and treating symptoms and complications as they arise. The only "cure" is liver transplantation, which may be an option when the disease progresses to cirrhosis and the liver function is affected.When some of the larger bile ducts become blocked, there is potential to open them with ERCP (endoscopic retrograde cholangiopancreatography) and balloon dilatation and/or stent placement. A gastroenterologist passes a thin fiberoptic scope with a camera into the mouth and threads it through the stomach into the bile ducts. Should a narrow duct be found, a balloon can be used to dilate the narrowing or a stent can be placed to keep the duct open. This is similar to how a cardiologist opens a blocked blood vessel in the heartIn some cases, surgery may be an option to remove some scarred and blocked bile ducts and having more normal bile ducts reconnected bypassing scarred areas of the bile ducts.