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Primary Sclerosing Cholangitis

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Overview

Primary sclerosing cholangitis (PSC) is characterised by inflammation and scarring of the bile ducts. Its pathophysiology is not understood as despite the presence of autoantibodies and the association with other autoimmune diseases, PSC does not behave similarly to other autoimmune diseases and does not respond to immunosuppressants.

Epidemiology

  • PSC is rare with a prevalence of 16 cases per 100,000
  • PSC is more common in men
  • Patients with PSC often present aged 40-50 years
  • PSC is associated with ulcerative colitis

Risk Factors

  • Male sex
  • Ulcerative colitis (UC) – around 75% of patients with PSC have UC
  • Family history

Presentation

Patients may be asymptomatic and have abnormal LFTs found incidentally. Other features can be:

Differential Diagnoses

Primary biliary cirrhosis (PBC)

  • PBC affects small interlobular bile ducts, PSC affects intrahepatic and extrahepatic ducts
  • PBC mainly affects women, PSC mainly affects men
  • PBC is not associated with ulcerative colitis
  • PBC is associated with anti-mitochondrial antibodies, but PSC is not

Investigations

  • LFTs:
    • Show cholestatic results, i.e. ALP and gamma-GT are more significantly increased compared to AST and ALT, which may be normal/slightly increased
  • Autoantibodies:
  • Abdominal ultrasound:
    • An initial test to rule out obstruction
  • Magnetic resonance cholangiopancreatography (MRCP):
    • Diagnostic test which shows intra- and/or extrahepatic structures

Management

Overview

No effective medical treatment is available. Treatment is mainly symptomatic and a liver transplant is the only option available for advanced disease. Cholestyramine may help with pruritus.

Complications

Prognosis

  • PSC is progressive and eventually leads to liver fibrosis and cirrhosis
  • Transplantation is not curative, and up to 1 in 5 develop recurrence in the graft

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