What is the management of primary sclerosing cholangitis (PSC)?

Management of Primary Sclerosing Cholangitis (PSC)

There is no proven effective medical therapy for primary sclerosing cholangitis, and management should focus on symptom control, surveillance for complications, and timely referral for liver transplantation when appropriate. 1, 2

Diagnosis and Initial Evaluation

• Diagnosis requires indirect (MRCP) or direct cholangiography (ERCP) 1
• Liver biopsy not routinely needed with typical cholangiographic findings 1
• All patients should be:
  • Screened for colitis with colonoscopy and biopsies 1
  • Tested for serum IgG4 levels to exclude IgG4-associated sclerosing cholangitis 1
  • Assessed for risk of osteoporosis 1

Medical Management

Ineffective Treatments

• Ursodeoxycholic acid (UDCA) is not recommended for routine treatment of PSC or prevention of colorectal cancer/cholangiocarcinoma 1, 2
• Corticosteroids and immunosuppressants are not indicated for classic PSC 1
  • Exception: Patients with overlapping autoimmune hepatitis or IgG4-related sclerosing cholangitis may benefit from corticosteroids 1

Symptom Management

• Pruritus:
  • First-line: Cholestyramine or similar bile acid sequestrants
  • Second-line: Rifampicin and naltrexone 1, 2
• Fatigue: Actively seek and treat alternative causes 1
• Nutrition: Low threshold for empirical replacement of fat-soluble vitamins (A, D, E, K) in advanced PSC 1, 2

Surveillance and Monitoring

• Annual clinical assessment and blood tests (more frequent for advanced disease) 2
• Malignancy surveillance:
  • Annual colonoscopic surveillance for patients with coexistent IBD 1
  • Annual ultrasound of gallbladder to screen for polyps/malignancy 1, 2
  • CA19.9 has low diagnostic accuracy and is not recommended for routine cholangiocarcinoma surveillance 1
• Endoscopic screening for esophageal varices in patients with cirrhosis/portal hypertension 1

Management of Complications

Dominant Strictures

• Definition: Stenosis with diameter ≤1.5 mm in common bile duct or ≤1 mm in hepatic duct 1
• Occurs in 45-58% of patients during follow-up 1
• Management approach:
  • ERCP only after expert multidisciplinary assessment 1
  • Mandatory pathological sampling of suspicious strictures 1
  • Biliary dilatation preferred over biliary stenting 1
  • Prophylactic antibiotics should be administered 1

Cholangiocarcinoma

• Occurs in 10-20% of PSC patients 3
• When suspected, referral for specialist multidisciplinary review is essential 1
• Initial investigation: Contrast-enhanced cross-sectional imaging 1
• Confirmatory diagnosis requires histology 1

Liver Transplantation

• Only proven life-extending therapy for end-stage PSC 3
• Required in approximately 40% of patients 3
• Excellent outcomes compared to other transplant indications, though disease can recur post-transplant 3
• Eligibility and referral should follow national guidelines 1

Special Considerations

• Pregnancy: Cirrhotic patients require preconception counseling and specialist monitoring due to higher risk of maternal and fetal complications 1
• Psychological support: Encourage participation in patient support groups 1, 2
• Inflammatory bowel disease: Present in 60-80% of PSC patients in Western countries 4
  • Annual colonoscopic surveillance recommended due to increased risk of colorectal cancer 1, 4

Pitfalls to Avoid

• Delaying referral for liver transplantation evaluation
• Using high-dose UDCA which may be potentially harmful 5
• Performing unnecessary ERCP without clear indication (risk of cholangitis)
• Missing surveillance for associated malignancies
• Failing to distinguish PSC variants (IgG4-associated cholangitis, overlap with autoimmune hepatitis) which may require different treatment approaches 5