Endoscopic Therapy for Primary Sclerosing Cholangitis with Dominant Bile Duct Strictures

Diagnostic Approach

Magnetic resonance cholangiography (MRC) should be the primary diagnostic modality for PSC, as recommended by the European Association for the Study of the Liver, rather than ERCP 1
ERCP should be considered before therapeutic intervention to confirm dominant strictures and perform ductal sampling to exclude cholangiocarcinoma, with a strength of evidence based on expert opinion 1
Cholangiocarcinoma should be suspected in any patient with worsening cholestasis, weight loss, raised serum tumor markers, and/or new or progressive dominant stricture, according to the European Society of Gastrointestinal Endoscopy 1

Balloon dilation alone should be the preferred initial treatment for dominant strictures in PSC, as recommended by the American Society for Gastrointestinal Endoscopy, with a strength of evidence based on moderate-quality evidence 2
Select a balloon caliber up to the maximum caliber of the ducts delimiting the stricture, typically 6-8 mm for hepatic ducts and up to 8 mm for common bile duct, based on expert opinion 3
Balloon dilations are usually repeated at intervals of 1-4 weeks until technical success, typically requiring 2-3 sessions, with a success rate of 80-90% 3
Technical success is defined as complete balloon inflation with no waist observed fluoroscopically, followed by unobstructed passage of contrast medium, as defined by the European Society of Gastrointestinal Endoscopy 3
This approach has shown lower complication rates (15% vs 54%) compared to stenting, with a strength of evidence based on high-quality evidence 2

If stenting is required, short-term stenting is preferred over long-term stenting to reduce complications, as recommended by the American College of Gastroenterology, with a strength of evidence based on low-quality evidence 2, 4
Long-term stenting is associated with higher risk of stent clogging and cholangitis, with a strength of evidence based on moderate-quality evidence 2
The European multicenter randomized DILSTENT trial was prematurely stopped due to significantly higher serious adverse event rates in the stent group compared to balloon dilation, with a strength of evidence based on high-quality evidence 4

Biliary sphincterotomy should not be performed routinely but considered on a case-by-case basis, as recommended by the European Society of Gastrointestinal Endoscopy, with a strength of evidence based on expert opinion 4
Sphincterotomy is particularly indicated after difficult cannulation in patients likely to require multiple ERCPs, with a strength of evidence based on low-quality evidence 4
In PSC, sphincterotomy has been associated with increased risk of short-term adverse events, but may be protective for subsequent ERCPs, with a strength of evidence based on moderate-quality evidence 4

Prophylactic antibiotics should be administered routinely before ERCP in patients with PSC, as recommended by the American Society for Gastrointestinal Endoscopy, with a strength of evidence based on high-quality evidence 1
Bile duct perforation rates are lower with balloon dilation (0.2% of procedures) compared to stenting, with a strength of evidence based on moderate-quality evidence 3

The American Association for the Study of Liver Diseases recommends endoscopic balloon dilatation as the first-line endoscopic therapy for patients with primary sclerosing cholangitis (PSC) and dominant bile duct strictures, with stenting reserved for strictures refractory to dilatation 5, 6

A dominant stricture in PSC is defined as a stenosis with a diameter of ≤1.5 mm in the common bile duct or ≤1 mm in the hepatic duct, occurring in 45-58% of PSC patients during follow-up 7
Dominant strictures can lead to biliary obstruction, causing symptoms and liver function deterioration 5

The European Association for the Study of the Liver recommends suspecting and evaluating for dominant strictures in patients with increasing serum bilirubin levels, worsening pruritus, progressive bile duct dilatation on imaging, cholangitis, or deteriorating liver biochemical indices 8
Diagnostic workup should include endoscopic retrograde cholangiography (ERC) to visualize and confirm dominant strictures, as well as brush cytology and/or endoscopic biopsy to exclude cholangiocarcinoma before any therapeutic intervention 8

The American College of Gastroenterology recommends endoscopic balloon dilatation as the first-line therapy for dominant strictures, with balloon dilatation shown to be effective alone 5
Stenting is reserved for strictures refractory to dilatation alone, with technical success rates of 91.9% and clinical success rates of 70.8% 5, 6

The American Association for the Study of Liver Diseases recommends treating bacterial cholangitis with antibiotics and biliary decompression, and considering prophylactic long-term antibiotics for recurrent bacterial cholangitis 6, 9
Refractory bacterial cholangitis should be evaluated for liver transplantation 9
Strictures refractory to endoscopic management should be considered for surgical therapy in selected patients without cirrhosis 10