PSC

• Primary sclerosing cholangitis is a chronic progressive inflammatory condition that affects the intra and extra-hepatic bile ducts leading to fibrosis and stricture formation
• Primary sclerosing cholangitis is more common in men aged 30-40. Most people with PSC also have ulcerative colitis.
• Often patients with Primary sclerosing cholangitis are asymptomatic at diagnosis, with the problem picked up on abnormal liver function tests. They may present with:

  • RUQ pain
  • Pruritus (itching)
  • Jaundice
  • Hepatomegaly
  • Splenomegaly
• Diagnosis of primary sclerosing cholangitis:

  • Raised ALP
  • Raise bilirubin - usually later in the disease
  • Antinuclear cytoplasmic antibody (ANCA) and antinuclear antibodies (ANA) - only present in 30% of patients
  • MRCP
  • Colonoscopy should be performed to assess for ulcerative colitis
• Management of primary sclerosing cholangitis:

  • ERCP may be used to treat dominant strictures
  • Liver transplant for advanced disease
  • Colestyramine for itching
  • Monitoring for complications such as cholangiocarcinoma, cirrhosis and oesophageal varices
• A secondary cholangiopathy refers to any condition causing bile duct damage and biliary obstruction. Numerous causes such IgG4-sclerosing cholangitis, cholangiocarcinoma or recurrent cholangitis.
• There is an increased risk of cholangiocarcinoma. With continued inflammation there is progressive liver scarring, development of cirrhosis and subsequent decompensation. Without liver transplantation, median survival following diagnosis is 10-12 years
• Symptoms:

  • Fatigue
  • Pruritus
  • Features of cholangitis
• Signs:

  • Hepatomegaly
  • Splenomegaly
  • Excoriations
  • Stigmata of chronic liver disease
• A formal diagnosis of PSC can be made based on the presence of cholestatic liver function tests (i.e. raised ALP and gGT) and typical appearance on magnetic resonance cholangiopancreatography (MRCP)
• Classic changes seen on MRCP:

  • Biliary stricturing: may be focal or diffuse. Majority of patients have strictures affecting both intra- and extrahepatic ducts
  • Biliary dilatation: may be intrahepatic and/or extrahepatic
• Liver transplantation is the principle treatment in patients with advanced liver disease secondary to PSC.
• There is a high incidence of colorectal cancer (CRC) in patients with PSC who also have coexistent IBD. Patients with IBD and PSC require annual surveillance colonoscopy.
• Complications:

  • Recurrent cholangitis
  • Cholangiocarcinoma
  • Chronic liver disease
  • Metabolic bone disease (e.g. osteoporosis)
  • Poor nutrition
  • Fatigue and depression
  • Pruritus