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 | | Figure 1. Location of the biliary tree in the body. |
Primary sclerosing cholangitis (PSC) is a chronic, usually progressive, stricturing disease of the biliary tree. Remissions and relapses characterize the disease course. Primary sclerosing cholangitis may remain quiescent for long periods of time in some patients; in most cases, however, it is progressive. The prevalence of primary sclerosing cholangitis in the United States is approximately 1–6 cases per 100,000 population. Most patients with primary sclerosing cholangitis are men (75%) with an average age of approximately 40 years at diagnosis. The overwhelming majority of patients affected with primary sclerosing cholangitis are Caucasian. The etiology is unknown but current opinion favors an immune cause. Management of this disease in the early stages involves the use of drugs to prevent disease progression. Endoscopic and surgical approaches are reserved for the time when symptoms develop. Liver transplantation may ultimately be required and offers the only chance for a complete cure. Patients with primary sclerosing cholangitis are at an increased risk for cholangiocarcinoma (10–15%).
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What is PSC?
Primary sclerosing cholangitis is a chronic fibrosing inflammatory process that results in the obliteration of the biliary tree and biliary cirrhosis. There is variability in the extent of involvement of the biliary system. The majority of patients with primary sclerosing cholangitis have underlying inflammatory bowel disease, namely ulcerative colitis or Crohn’s disease. Patients with primary sclerosing cholangitis are more likely to have ulcerative colitis than Crohn’s disease (85% versus 15%), with approximately 2.5–7.5% of all ulcerative colitis patients having primary sclerosing cholangitis. The strictures are located in both the intrahepatic and extrahepatic ducts in more than 80% of the patients, but about 10% of these patients have intrahepatic strictures only while less than 5% will have only extrahepatic strictures (Figure 2).
Symptoms
 | | Figure 3. Symptoms of advanced disease. |
Most patients with primary sclerosing cholangitis have no symptoms. These patients are usually diagnosed by the detection of abnormal biochemical tests of liver function on routine blood testing. Patients may remain asymptomatic for many years despite the presence of advanced disease.
When symptoms develop they are a result of obstruction to bile flow and include jaundice, itching, right upper quadrant abdominal pain, fever, and chills. Symptoms may also include weight loss and fatigue. The development of symptoms usually suggests the presence of advanced disease (Figure 3).
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