Intrahepatic cystic biliary dilatation constitutes a significant prognostic factor in patients with primary sclerosing cholangitis
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To evaluate the prognostic value of cystic dilatation (CD) of the intrahepatic biliary ducts in patients with primary sclerosing cholangitis (PSC).
A single-center cohort of 205 patients with PSC from 2003 to 2016 was analysed. CD was defined by quantitative and qualitative criteria. Radiological and clinical courses were assessed. A Kaplan-Meier analysis was used to estimate cumulative survival without liver transplantation (LT) from the date of PSC diagnosis. A log-rank test was performed to compare survival time of PSC patients with and without CD.
A total of 15 (7.3%) PSC patients (12 males) with a median age of 23 years at diagnosis had CD. Five patients had one CD; seven patients had two or three CDs; and three patients had diffuse CD. CDs ranged in small diameter size from 12 to 32 mm. Radiological evolution of CD was markedly variable. However, a radiological worsening of PSC over time was observed in all patients. The clinical course was characterized by the occurrence of complications in most patients. Half of the patients with CD underwent LT at a median time of 40 months from diagnosis of CD and the median survival time from PSC diagnosis was significantly lower than in PSC without CD (10.7 vs. 23.4 years; HR 3.8, 95% confidence interval: 1.7–8.3, p = 0.001).
CD in PSC is an unusual condition that mostly affects young patients. It is characterized by a rapid, unfavorable course and constitutes a significant prognostic factor.
• Cystic dilatation of the intrahepatic biliary ducts affects young patients with primary sclerosing cholangitis and is characterized by a markedly variable radiological evolution.
• Biliary wall inflammation, found in explanted livers, could be a key feature in the pathogenesis of cystic dilatation.
• Cystic dilatation of the intrahepatic biliary ducts is characterized by an unfavorable course and constitutes a significant prognostic factor of primary sclerosing cholangitis.
KeywordsCholangitis sclerosing Cholangiography Magnetic resonance imaging
Endoscopic retrograde cholangiopancreatography
End-stage liver disease
Inflammatory bowel disease
Magnetic resonance cholangiography
Magnetic resonance imaging
Mayo risk score
Primary sclerosing cholangitis
Systemic lupus erythematous
This work was funded by “Fonds CSP” and NC was granted the EASL Sheila Sherlock fellowship 2017-2018.
Compliance with ethical standards
The scientific guarantor of this publication is Lionel Arrivé M.D.
Conflict of interest
The authors of this manuscript declare no relationships with any companies whose products or services may be related to the subject matter of the article.
Statistics and Biometry
One of the authors, Christophe Corpechot, has significant statistical expertise.
Written informed consent was waived by the Institutional Review Board.
Institutional Review Board approval was obtained.
• Performed at one institution
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