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What is the deal Primary Sclerosing Cholangitis?

It is a chronic liver disease characterized by a progressive course of cholestasis with inflammation and fibrosis of the intrahepatic and extrahepatic bile ducts. The underlying cause of the inflammation is believed to be that of autoimmunity. The condition may lead to cirrhosis of the liver with hypertension and end-stage liver disease. Primary sclerosing cholangitis has been considered a rare disease. The disease may occur alone but frequently exists in association with inflammatory diseases of the colon, such as chronic ulcerative colitis. Primary sclerosing cholangitis affects both sexes but is more common in men. PSC is most often seen between the ages of 30 and 45. In primary sclerosing cholangitis, inflammation causes scars within the bile ducts. These scars make the ducts hard and narrow and gradually cause serious liver damage.

What are the symptoms associated with PSC?

Many people with PSC are not affected with symptoms, especially in the early stages of the disease. However when they occur, the most common ones are fatigue, pruritus, or itching of the skin, and jaundice, a yellowing of the skin and eyes. As the disease continues, the bile ducts may become infected, which can lead to fever, abdominal pain and chills.

Causes :

An immune system's reaction to any kind of infection might trigger the disease in people who are genetically exposed to it. A large proportion of people with primary sclerosing cholangitis is also affected with inflammatory bowel disease.

Primary sclerosing cholangitis and inflammatory bowel disease don't always appear at the same time, though. In some cases, primary sclerosing cholangitis is present for years before inflammatory bowel disease occurs. If primary sclerosing cholangitis is diagnosed, it's important to look for inflammatory bowel disease because there is a greater risk of colon cancer.

Diagnosis :

PSC is generally diagnosed on the basis of having at least two of three clinical:

    serum alkaline phosphatase (ALP) > 1.5x the upper limit of normal;

    cholangiography demonstrating biliary strictures or irregularity consistent with PSC; and,

    liver histology (if available).

Historically, a cholangiogram would be obtained via endoscopic retrograde cholangiopancreatography, which typically reveals beading of the bile ducts inside or outside the liver. However, the preferred option for diagnostic cholangiography, for its non-invasive yet highly accurate nature, is magnetic resonance cholangiopancreatography. MRCP has unique strengths, including high spatial resolution, and can even be used to visualize the biliary tract of small animal models of PSC

To make your bile ducts visible on an X-ray, your doctor uses a flexible tube passed down your throat to inject dye into the area of your small intestine where your bile ducts empty. An ERCP is the test of choice if signs and symptoms persist despite no abnormalities on an MRI. An ERCP is often the initial test if you're unable to have an MRI because of a metal implant in your body.

How is Primary Sclerosing Cholangitis treated?

There is no particular cure or specific treatment associated with PSC. The itching associated with the disease can be reduced with the proper dosage of medication, and antibiotics are used to treat bile duct infections when they occur. Most people with PSC must take vitamin supplements. In some cases, bile duct surgery or endoscopy may be useful to temporarily improve bile flow.

When the disease has progressed to liver failure, liver transplantation may be considered.  Liver transplantation is successful in 85 to 90 per cent of adult patients.

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