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)

References
Hunter, M. et al. Evaluating distinctive features for early diagnosis of primary sclerosing cholangitis overlap syndrome in adults with autoimmune hepatitis. Ulster Med J. 2007; 80(1): 15-18. 
Nguyen, D. and Lazaridis, K. Ulcerative colitis and an abnormal cholangiogram. Cleve Clin J Med. 2011; 78(5): 306-311.
Silveira, M. and Lindor, K. Primary sclerosing cholangitis. Can J Gastroenterol. 2008; 22(8): 689-698.