Primary sclerosing cholangitis
Primary sclerosing cholangitis (PSC) is characterised by stricturing of intrahepatic or extrahepatic bile ducts, resulting in chronic cholestasis. It is associated with inflammatory bowel disease (IBD) in 70 to 80% of patients.
Magnetic resonance cholangiopancreatography (MRCP) is preferred to endoscopic retrograde cholangiopancreatography (ERCP) for diagnosis, because it is less invasive and has no risk of introducing infection.
An important differential diagnosis of primary sclerosing cholangitis is immunoglobulin G4–related sclerosing cholangitis; see Immunoglobulin G4–related disease for diagnosis and management.
Ursodeoxycholic acid improves liver biochemistry in adults with primary sclerosing cholangitis; however, it has not been shown to improve survival. The drug is prescribed by some specialists despite limited evidence for benefit. If ursodeoxycholic acid is used, standard doses should be prescribed; high doses have been associated with an increased risk of serious adverse effects.
Primary sclerosing cholangitis can be associated with acute (ascending) cholangitis, which requires antibiotic therapy—see Acute cholangitis for antibiotic regimens. In patients with frequent recurrences of acute cholangitis, long-term antibiotic therapy may be needed. The decision to use long-term antibiotic therapy and choice of antibiotic require expert multidisciplinary involvement; see also Management of recurrent cholangitisBSG 2019EASL 2022. Recurrent cholangitis requiring long-term antibiotic therapy can be an indication for liver transplantation.
Patients with primary sclerosing cholangitis who have new symptoms or changes in liver biochemistry should be investigated for a dominant biliary stricture or cholangiocarcinoma, initially with magnetic resonance cholangiopancreatography (MRCP). Some patients require endoscopic retrograde cholangiopancreatography (ERCP) for investigation and management of dominant biliary strictures. ERCP with brush cytology can be used to investigate for cholangiocarcinoma.
Complications of primary sclerosing cholangitis include dyslipidaemia, fat-soluble vitamin deficiency, metabolic bone disease and itch.
- Measure the patient’s blood lipid levels, fat-soluble vitamin concentrations and bone mineral density.
- Treat dyslipidaemia with lipid-lowering drugs if the patient has cardiovascular risk factors (see Lipid modification).
- Prescribe fat-soluble vitamins if indicated (see Fat-soluble vitamin deficiencies for more information).
- For discussion about bone health, see Bone health in patients with cirrhosis.
- For management of itch, see Cholestatic itch in patients with liver disease.
Surveillance for malignancy is recommended for patients with primary sclerosing cholangitis, as follows:
- annual colonoscopy for patients with primary sclerosing cholangitis and inflammatory bowel disease, who have an increased risk of colorectal cancer
- annual ultrasound scan of the gallbladder
- 6-monthly surveillance for hepatocellular carcinoma in patients with primary sclerosing cholangitis who have cirrhosis.