What is the recommended first-line endoscopic therapy for patients with primary sclerosing cholangitis (PSC) and dominant bile duct strictures?

Endoscopic Therapy for Primary Sclerosing Cholangitis with Dominant Bile Duct Strictures

For patients with primary sclerosing cholangitis (PSC) and dominant bile duct strictures, endoscopic balloon dilatation is the recommended first-line endoscopic therapy, with stenting reserved only for strictures refractory to dilatation. 1

Understanding Dominant Strictures in PSC

A dominant stricture in PSC is defined as:

• Stenosis with a diameter of ≤1.5 mm in the common bile duct or ≤1 mm in the hepatic duct 1
• Occurs in 45-58% of PSC patients during follow-up 1
• Can lead to biliary obstruction, causing symptoms and liver function deterioration 1

Diagnostic Approach for Dominant Strictures

When to suspect and evaluate for dominant strictures:

• Increasing serum bilirubin levels 1
• Worsening pruritus 1
• Progressive bile duct dilatation on imaging 1
• Cholangitis 1
• Deteriorating liver biochemical indices 1

Diagnostic workup should include:

• Endoscopic retrograde cholangiography (ERC) to visualize and confirm dominant strictures 1
• Brush cytology and/or endoscopic biopsy to exclude cholangiocarcinoma before any therapeutic intervention 1

Recommended Endoscopic Management Algorithm

1. First-line therapy: Endoscopic balloon dilatation 1, 2

   • Balloon dilatation has been shown to be effective alone 1
   • Technical success rates of 96.8% 2
   • Clinical success rates of 86.5% 2
   • Lower overall adverse event rates (11.2%) compared to stenting 2

2. Second-line therapy: Balloon dilatation with stenting 1

   • Reserved for strictures refractory to dilatation alone 1
   • Technical success rates of 91.9% 2
   • Clinical success rates of 70.8% 2
   • Higher adverse event rates (26.9%) compared to balloon dilatation alone 2

3. Alternative approach: Percutaneous biliary dilatation 1

   • Consider when endoscopic approach is unsuccessful 1
   • Associated with increased morbidity but similar efficacy to endoscopic approach 1

Clinical Outcomes and Benefits

Endoscopic therapy for dominant strictures has demonstrated:

• Improvement in clinical symptoms and laboratory parameters 3
• Significant reductions in serum bilirubin (73%), alkaline phosphatase (46%), and liver enzymes 3
• Potentially improved survival compared to predicted natural history 4
• Observed 5-year survival rates significantly better than predicted by Mayo Risk Score in some studies 1

Important Considerations and Precautions

• Malignancy screening: Always perform brush cytology and/or endoscopic biopsy before therapeutic intervention to exclude cholangiocarcinoma 1
• Antibiotic prophylaxis: Administer perioperative antibiotics to prevent cholangitis when injecting contrast into obstructed ducts 1
• Cholangiocarcinoma risk: Dominant strictures are associated with increased risk of cholangiocarcinoma (26% in patients with dominant strictures vs. 0% in those without) 5
• Procedure-related complications: Overall complication rates for endoscopic procedures range from 1-7.3% 5, 4

Management of Complications

• Bacterial cholangitis: Treat with antibiotics and biliary decompression 1
• Recurrent bacterial cholangitis: Consider prophylactic long-term antibiotics 1
• Refractory bacterial cholangitis: Evaluate for liver transplantation 1
• Strictures refractory to endoscopic management: Consider surgical therapy in selected patients without cirrhosis 1

Follow-up Recommendations

• Routine endoscopic follow-up after treatment 3
• Monitor for clinical improvement, laboratory parameters, and cholangiographic findings 3
• Vigilance for development of cholangiocarcinoma, especially within the first months after PSC diagnosis 5