What is the management plan for a patient diagnosed with primary sclerosing cholangitis (PSC)?

Management of Primary Sclerosing Cholangitis (PSC)

The management of primary sclerosing cholangitis requires expert multidisciplinary care with regular monitoring, symptom management, and surveillance for complications, as no medical therapy has been proven to halt disease progression. 1

Initial Management and Referral

• Patients with symptomatic, evolving, or complex disease should be referred for expert multidisciplinary assessment 1
• Patients with early, stable disease can be managed in general clinics with at least annual clinical assessments 1
• Consider referral to centers participating in clinical trials for eligible patients 1

Medical Management

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

Symptom Management

• Pruritus:

  • First-line: Cholestyramine or similar bile acid sequestrants 1
  • Second-line: Rifampicin and naltrexone 1

• Fatigue:

  • Actively seek and treat alternative causes 1

• Nutritional support:

  • Low threshold for empirical replacement of fat-soluble vitamins (A, D, E, K) in advanced PSC 1
  • Risk assessment for osteoporosis with treatment according to national guidelines 1

Surveillance and Monitoring

1. Regular monitoring:

   • At minimum, annual clinical assessment and blood tests 1
   • More frequent evaluation for advanced disease 1

2. Colonoscopy:

   • All patients should be screened for colitis at diagnosis 1
   • Annual colonoscopic surveillance for patients with coexistent IBD 1

3. Imaging surveillance:

   • Annual ultrasound of gallbladder to screen for polyps/malignancy 1
   • Non-invasive investigations (MRCP, dynamic liver MRI, contrast CT) for new/changing symptoms or abnormal laboratory values 1

4. Variceal screening:

   • Endoscopic screening for esophageal varices in patients with cirrhosis and/or portal hypertension 1, 2

5. Cancer surveillance:

   • CA19.9 has low diagnostic accuracy and is not recommended for routine cholangiocarcinoma surveillance 1
   • When cholangiocarcinoma is suspected, contrast-enhanced cross-sectional imaging is the initial preferred investigation 1

Endoscopic Management

• ERCP should be performed only after expert multidisciplinary assessment justifies intervention 1, 2
• For dominant strictures:
  • Pathological sampling of suspicious strictures is mandatory 1
  • Biliary dilatation is preferred over biliary stenting 1, 2
  • Prophylactic antibiotics should be given for ERCP procedures 1

Liver Transplantation

• PSC is a well-recognized indication for liver transplantation 1, 3
• Transplantation is the only life-extending therapeutic approach for end-stage PSC, ultimately required in approximately 40% of patients 3
• Eligibility and referral should be assessed according to national guidelines 1
• Post-transplant outcomes are excellent compared to other indications, though disease recurrence can occur 3

Management of Complications

1. Dominant strictures:

   • Require expert multidisciplinary review 1
   • Suspicious strictures need pathological sampling to rule out cholangiocarcinoma 1

2. Cholangiocarcinoma:

   • When suspected, immediate referral for specialist multidisciplinary meeting review 1
   • Diagnosis relies on histology with tissue sampling approach guided by MDM review 1

3. Gallbladder abnormalities:

   • Polyps identified on ultrasound should be directed to specialist hepatopancreaticobiliary MDM 1

4. Pregnancy considerations:

   • Cirrhotic patients require preconception counseling and specialist monitoring due to higher risk of maternal and fetal complications 1

Patient Support

• Encourage participation in patient support groups 1
• Address psychological aspects including anxiety, depression, and social isolation 1

Common Pitfalls and Caveats

• Avoid unnecessary ERCP procedures due to risk of complications; use only after multidisciplinary assessment 1
• Do not rely on UDCA for disease modification despite its widespread use in clinical practice 1, 2
• Do not overlook screening for IBD in all PSC patients, even if asymptomatic 1
• Recognize that PSC has an unpredictable course with serious complications requiring lifelong surveillance 2
• Be vigilant for cholangiocarcinoma development, which accounts for a large proportion of mortality in PSC 3