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
- Weight loss
- Right upper quadrant (RUQ) pain
- Recurrent biliary infections
- 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
- Liver function tests
- 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
- Ultrasound liver
- 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)
- 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
Common primary sclerosing cholangitis (PSC) exam questions for medical students, finals, OSCEs and MRCP PACES
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