Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease, likely autoimmune.

It most commonly affects men (3:1) and mean age of diagnosis is 40.

At presentation up to 55% of patients are asymptomatic.  Possible symptoms include:
  • pruritis
  • fatigue
  • abdominal pain
  • jaundice
Investigations:
  • LFTs - raised ALP, mild to moderate increases in AST/ALT
  • Autoantibodies
    • ANCA positive in 80% - but not specific
    • ANA - 20-60%, again not specific
    • SMA - 20-60%, not specific
    • (AMA is rarely positive - far more associated with PBC)
  • MRCP - gold standard
  • ERCP
    • shows strictures of biliary tree with characteristic 'beaded' appearence
  • Liver biopsy
    • not necessarily helpful
    • may reveal concentric periductal fibrosis (='onion skinning')

Complications
  • cholangiocarcinoma - up to 30%
  • colorectal cancer

Associations:
  • 80% of patients with PSC have ulcerative colitis (although only 4% of patients with UC have PSC)
  • 7-50% of patients with PSC also have autoimmune hepatitis
Treatment:
  • ursodeoxycholic acid
  • colestyramine or rifampicin for pruritus
  • liver transplant
    • but up to 40% recurrence of PSC in organ
    • median time from diagnosis to transplant needed: 18 years
  • ERCP to stent strictures
  • (yearly colonoscopy if UC)

0 comments:

Post a Comment

Comments? Corrections? Thoughts?