Lecture: Primary sclerosing cholangitis

This lecture reviewed the diagnosis and management of primary sclerosing cholangitis (PSC) and represented the newest AASLD practice guidelines, published February 2010 in Hepatology (see link to article below).  PSC is an autoimmune disease primarily of the large intra or extrahepatic bile ducts.  However, when a patient presents with the typical biochemical profile, clinical and histological features but has a normal cholangiogram, they are labeled as having small duct PSC.

The diagnostic accuracy is roughly equivalent for MRCP and ERCP, though the former has fewer complications.  When radiographic diagnosis is in doubt, an antibody profile can be helpful: ANA, ANCA, anti-cardiolipin Ab, anti-endothelial cell Ab and anti-smooth muscle cell Ab can be variably elevated.

After a diagnosis is made, an IgG-4 serum level should be obtained, to rule out autoimmune pancreatitis, which is often confused with PSC because it presents with biliary strictures.  A colonoscopy should always be performed, because over 75% of cases of PSC come in the setting of ulcerative colitis (UC) or Crohn’s disease.  When a large (ie. dominant) stricture is identified, a workup for cholangiocarcinoma must be undertaken.

Interestingly, the guidelines did not support the use of ursodeoxycholic acid for the treatment of PSC.  What we do know is that high doses (28-30 mg/kg) increase morbidity and mortality, and lower doses (15 or 20-22 mg/kg) may not have any benefit.  The crowd’s reaction was mixed, and many seem willing to continue to prescribe Urso at a moderate dose, particularly in PSC patients with early stage disease.

Other topics discussed include management of gallbladder polyps, recurrent cholangitis and referral for liver transplantation.  For the full article, published February 2010 in Hepatology, click on the link below:

http://www.aasld.org/practiceguidelines/Documents/Practice%20Guidelines/PSC_2-2010.pdf

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