Prognostic Significance of Cystic Biliary Dilatation in Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a chronic, progressive disease characterized by inflammatory and fibrotic strictures in the intrahepatic and/or extrahepatic bile ducts. Typical cholangiographic findings include multifocal, annular, short bile duct strictures with minimal upstream dilatation. However, in some patients, this dilatation progresses to cystic dimensions. The current study aimed to investigate whether the clinical features and prognosis of patients with cystic dilatation (CD) of the bile ducts differ from those of patients with classic cholangiographic PSC.

Demographic data, laboratory and clinical findings, and prognostic outcomes of patients with CD of the bile ducts who underwent endoscopic retrograde cholangiopancreatography (ERCP) and/or percutaneous transhepatic biliary drainage at the hospital between 2017 and 2023 were compared with those of patients without CD.

Of the 37 patients, 5 (13.5%) had cystic biliary dilatation. Median follow-up was 24 months (range, 12-62) in the CD group and 20 months (range, 8-52) in the non-cystic group. Patients with CD had a higher frequency of cholangitis (median attacks per patient, 1.6 vs. 0.3; P < .001), a greater proportion of recurrent cholangitis (4/5 [80%] vs. 7/32 [21.8%]; P = .038), an increased risk of end stage liver disease (3/5 [60%] vs. 5/32 [15.6%]; P = .025), and higher mortality (1/5 [20%] vs. 2/32 [6.25%]; P = .045). Median transplant free survival was significantly shorter in the cystic group (7.1 years [95% CI, 1-9] vs. 19.5 years; P = .017).

Cystic dilatation appears to be a clinically relevant prognostic feature in patients with primary sclerosing cholangitis, however, confirmation in larger studies is warranted.