The Challenges Surrounding Primary Sclerosing Cholangitis

A Lack of Clarity Surrounding Treatment

There is no proven medical therapy for PSC. Low- and high-dose trials of ursodeoxycholic acid have not improved outcomes or reduced the need for liver transplantation, though the clinical benefit of low-dose ursodeoxycholic acid was suggested by one study showing worsening of liver tests following drug withdrawal.

The AASLD guidelines suggest:

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  In adult patients with PSC, ursodeoxycholic acid should not be used as medical therapy; and
  
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  In adult patients with PSC and overlap syndrome with autoimmune hepatitis, corticosteroids and other immunosuppressive agents can be used for medical therapy.
  

Up to 50% of PSC patients will develop major biliary strictures and present with worsening jaundice, increasing liver tests, bacterial cholangitis, or other signs of clinical decompensation. When a dominant biliary stricture of the common bile duct, common hepatic duct, or intrahepatic duct is identified, the potential diagnosis of cholangiocarcinoma should be considered. Cholangiocarcinoma should be excluded prior to duct dilation and/or stenting. Fluorescence in situ hybridization to assess for aneuploidy in brushings from strictures is a complementary technique with cytology for the diagnosis of cholangiocarcinoma. For benign dominant strictures, balloon dilation and short-term stenting appears best. Long-term stenting of benign dominant strictures in patients with PSC may be associated with increased risk for complications.

For those with dominant stricturing disease, the AASLD guidelines suggest:

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  Patients with worsening pruritus, increases in laboratory signs of cholestasis, bacterial cholangitis, or progressive bile duct dilation on imaging studies should have an ERC to exclude dominant stricture;
  
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  Brush cytology and/or endoscopic biopsy of a dominant stricture should be performed before endoscopic therapy is undertaken;
  
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  Patients with dominant strictures from PSC should be initially managed with endoscopic dilation with or without stenting;
  
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  Antibiotics should be administered in conjunction with endoscopic or percutaneous bile duct dilation and/or stenting;
  
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  If an endoscopic approach is unsuccessful, biliary dilation and stenting at percutaneous cholangiography can be considered; and
  
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  Patients with dominant strictures from PSC refractory to endoscopic or percutaneous management should be considered for surgical treatment if concurrent cirrhosis is not present.
  

Liver transplantation can be considered for PSC patients with decompensated end-stage liver disease, early-stage cholangiocarcinoma, or intractable symptoms interfering with quality of life, such as severe pruritus or recurring bacterial cholangitis. The 5-year survival following transplantation of PSC patients is approximately 85%. Up to 20% of patients may develop recurrent sclerosing cholangitis in their new liver allograft. The clinical course of inflammatory bowel disease following liver transplantation is variable, and annual surveillance colonoscopy must be continued.

The AASLD guidelines suggest:

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  Liver transplantation should be used as a treatment modality for PSC patients with advanced decompensated liver disease; and
  
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  Other causes of biliary strictures in the posttransplant setting should be excluded before making a diagnosis of recurrent PSC.