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­­Primary Sclerosing Cholangitis (PSC)


Definition of primary sclerosing cholangitis

  • Idiopathic disorder characterised by inflammation, fibrosis and structuring of the intra and extra-hepatic bile ducts.


 Epidemiology of primary sclerosing cholangitis

  • Male:Female = 2:1
  • Usually presents from 20-30 years old onward but can present earlier
  • Studies show a prevalence varying from 0.2 to 8.5 per 100,000
  • Higher prevalence in areas where ulcerative colitis is more common


 Aetiology of primary sclerosing cholangitis

  • PSC is strongly associated with inflammatory bowel disease (IBD)
    • Most studies show an IBD prevalence of 60-80% in PSC patients
    • The commonest type is ulcerative colitis (UC): present in up to 80% of those with IBD and PSC.
  • The underlying cause is unknown but there is thought to be an autoimmune component with a higher prevalence of certain HLA alleles (A1, B8, DR3).


 Presentations of primary sclerosing cholangitis

  • History:
    • Fatigue
    • Pruritus
    • Weight loss
    • Right upper quadrant (RUQ) pain
    • Recurrent biliary infections
  • Examination:
    • Jaundice
    • Hepatomegaly
    • Features of chronic liver disease
      • Dupuytren’s contracture; palmar erythema; gynaecomastia; spider naevi; ascites; cachexia
    • Features of IBD
      • Stoma; abdominal scars; mouth ulcers; erythema nodosum

 Differential diagnosis of primary sclerosing cholangitis

  • Any other cause of chronic liver disease (see chronic liver disease section)


 Investigation of primary sclerosing cholangitis

  • Blood tests:
    • Liver function tests
      • Usually show an elevated alkaline phosphatase, bilirubin and transaminases but these can all be in the normal ranges
    • Autoimmune profile
      • A range of autoantibodies can be present including ANCA , SMA and ANA
      • AMA (antimitochondrial) are usually absent
    • Full liver screen (see chronic liver disease section) to rule out other causes of cirrhosis
  • Imaging:
    • Ultrasound liver
      • Not usually diagnostic and can be normal. Gallstones and gallbladder thickening can be seen.
    • Magnetic resonance cholangiopancreatography (MRCP)
      • This has become the diagnostic imaging of choice and is has none of the associated morbidity of ERCP.
      • Sensitivity > 80% and specificity >87% for PSC diagnosis
  • Liver biopsy:
    • A biopsy is not needed for diagnosis of PSC.
    • Histological changes are often non-specific, especially in the early stages.
    • Liver biopsy is performed if MRCP and subsequent ERCP are non-diagnostic.


 Diagnosis of PSC (American Association of Liver Disease guidelines)

  • Cholestatic liver biochemistry
  • Cholangiography (MRCP or ERCP) showing characteristic changes to bile ducts including segmental strictures and dilatations


 Initial management of primary sclerosing cholangitis

  • Anti-pruritics: as per management in PBC (see PBC pages)
  • Vitamin A,D,E and K replacement
    • Vitamin D and Calcium replacement. Calcium 1g and vitamin D 800 IU daily
  • If bacterial cholangitis
    • Antibiotics plus therapeutic drainage of the obstruction
    • Sometimes patients require long-term prophylactic antibiotics if they are suffering recurrent infections
  • Stenting/drainage of strictures
    • Patients who are symptomatic from strictures with jaundice, cholangitis, RUQ pain or worsening liver function tests are candidates for intervention.
    • Endoscopic balloon dilatation, sphincterotomy and stent placement should be performed initially.
    • If endoscopic therapy is unsuccessful then biliary dilatation and/or stent placement should be attempted percutaneously.

Full guidelines on treatment can be found here: AASLD Guidelines – PSC

  • Orthotopic liver transplant
    • Transplantation criteria are the same for PSC patients as for all patients with chronic liver disease and also include recurrent cholangitis, intractable pruritus and limited cholangiocarcinoma.
    • Outcomes are good with a 5-year survival rate of 85%.

 Further management of primary sclerosing cholangitis

  • PSC patients with cirrhosis should be managed as for all chronic liver disease patients (see pages on chronic liver disease).


 Complications of primary sclerosing cholangitis

  • Biliary infections
  • Cirrhosis and associated complications (see pages on decompensated chronic liver disease)
  • Cholangiocarcinoma
    • Patients with PSC have a higher risk for cholangiocarcinoma. Studies show a 10-year cumulative incidence of 7-9%.
    • Any PSC patient whose liver biochemistry deteriorates should be investigated for a possible underlying cholangiocarcinoma.
  • Colorectal carcinoma
    • Patients with PSC and UC are at higher risk of developing colorectal carcinoma and should undergo colonoscopic surveillance 1-2 yearly from PSC diagnosis


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Common primary sclerosing cholangitis (PSC) exam questions for medical students, finals, OSCEs and MRCP PACES


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Perfect revision for medical students, finals, OSCEs and MRCP PACES