Primary sclerosing cholangitis (psc)

Abdomen | Gastroenterology | Primary sclerosing cholangitis (psc) (Disease)


Description

Primary sclerosing cholangitis (PSC) is a chronic liver disease caused by progressive inflammation and scarring of the bile ducts of the liver. The inflammation impedes the flow of bile to the gut, which can ultimately lead to liver cirrhosis, liver failure and liver cancer.

Inflammation damages bile ducts both inside and outside of the liver. The resulting scarring of the bile ducts blocks the flow of bile, causing cholestasis. Bile stasis and back-pressure induces proliferation of epithelial cells and focal destruction of the liver parenchyma, forming bile lakes.

Causes and Risk factors

Chronic biliary obstruction causes portal tract fibrosis and ultimately biliary cirrhosisliver failure and liver failure. The underlying cause of the inflammation is believed to be autoimmunity and more than 80% of those with PSC have ulcerative colitis.

Diagnosis and Treatment

Tests to determine the diagnosis and the extent of disease include: ERCP (endoscopic retrograde cholangiopancreatography), PTC (percutaneous transhepatic cholangiogram), liver biopsy, and/or MRCP (abdominal MRI).

Endoscopic balloon dilation and stenting of strictures may relieve symptoms. Antibiotics are prescribed for co-existing infections. The definitive treatment is liver transplant. ...