Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2019 Mar;29(3):1460-1468.
doi: 10.1007/s00330-018-5697-3. Epub 2018 Aug 29.

Intrahepatic cystic biliary dilatation constitutes a significant prognostic factor in patients with primary sclerosing cholangitis

Affiliations

Intrahepatic cystic biliary dilatation constitutes a significant prognostic factor in patients with primary sclerosing cholangitis

Laetitia Nguyen et al. Eur Radiol. 2019 Mar.

Abstract

Aims: To evaluate the prognostic value of cystic dilatation (CD) of the intrahepatic biliary ducts in patients with primary sclerosing cholangitis (PSC).

Methods: 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.

Results: 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).

Conclusions: 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.

Key points: • 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.

Keywords: Cholangiography; Cholangitis; Magnetic resonance imaging; sclerosing.

PubMed Disclaimer

Similar articles

Cited by

References

    1. Endoscopy. 1999 Feb;31(2):200-3 - PubMed
    1. AJR Am J Roentgenol. 2002 Oct;179(4):1053-7 - PubMed
    1. Radiographics. 2006 Nov-Dec;26(6):1603-20 - PubMed
    1. AJR Am J Roentgenol. 2008 Jul;191(1):221-7 - PubMed
    1. World J Gastroenterol. 2008 Nov 21;14(43):6748-9 - PubMed

LinkOut - more resources