Primary sclerosing cholangitis | Canada| PDF | PPT| Case Reports | Symptoms | Treatment

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Primary Sclerosing Cholangitis

  • Primary sclerosing cholangitis

    Disease pathophysiology:

    The pathophysiology of PSC is unclear, but there is evidence suggesting an autoimmune component to the disease. There is also a genetic predisposition, with an increased prevalence of HLA-B2 and DR3 in patients with PSC. Other proposed causes include chronic portal bacteremia, cytotoxic bile acids, and viral infections.

  • Primary sclerosing cholangitis

    Disease statistics: 

    Currently, no medical therapy has been shown to be beneficial in PSC. A 2-year randomized, controlled trial using UDCA at a dose of 12 to 15 mg/kg/d in patients with PSC was associated with improved liver tests, however there was no beneficial effect on survival, liver histology, cholangiographic appearance or symptoms.

  • Primary sclerosing cholangitis

    Disease treatment:

    Liver transplantation is effective for patients who have evidence of end-stage liver disease or who have recurrent bouts of cholangitis that cannot be controlled with dilation of a dominant stricture. Unfortunately, PSC recurs in 15% to 20% of cases, and recurrence is often associated with loss of the graft. Currently, no medical therapy has been shown to be beneficial in PSC. 

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