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
Review
. 2024 Jan 7;10(1):47-65.
doi: 10.3390/tomography10010005.

Primary Sclerosing Cholangitis: Diagnostic Criteria

Nora Cazzagon  1   2 , Samantha Sarcognato  3 , Elisa Catanzaro  1   2 , Emanuela Bonaiuto  1 , Matteo Peviani  1   2 , Francesco Pezzato  1   2 , Raffaella Motta  4   5
Affiliations
Review

Primary Sclerosing Cholangitis: Diagnostic Criteria

Nora Cazzagon et al. Tomography. .

Abstract

Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by inflammation and fibrosis of intra- and/or extrahepatic bile ducts leading to the formation of multifocal strictures alternated to bile duct dilatations. The diagnosis of the most common subtype of the disease, the large duct PSC, is based on the presence of elevation of cholestatic indices, the association of typical cholangiographic findings assessed by magnetic resonance cholangiography and the exclusion of causes of secondary sclerosing cholangitis. Liver biopsy is not routinely applied for the diagnosis of large duct PSC but is mandatory in the case of suspicion of small duct PSC or overlap with autoimmune hepatitis.

Keywords: PSC; chronic cholestasis; histological scoring system; liver biopsy; magnetic resonance cholangiography; magnetic resonance imaging; rare liver disease.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflicts of interest related to this study.

Figures

Figure 1
Figure 1
Primary sclerosing cholangitis. The 3D-MRC shows the presence of multiple strictures and dilatation of intra- and extrahepatic bile ducts. The common bile duct is severely stenotic in all its length.
Figure 2
Figure 2
Intraductal stones in PSC. T1w pre-contrast sequence showing the presence of a hyperintense calculus in a dilated intrahepatic bile duct in the posterior segment.
Figure 3
Figure 3
Cystic dilatations (CD) of intrahepatic bile ducts in PSC patients. MRCP sequence showing diffuse cystic dilatations of intrahepatic bile duct (A). MRI showing a large CD of intrahepatic bile duct dilatation localized between S5 and S6 filled with stones in T2 w images (B), T1w fat sat pre-contrast (C) and after Gd-EOB-DTPA (portal phase) showing wall thickening and enhancement of the CD (D).
Figure 4
Figure 4
Progressive parenchymal atrophy in PSC in the hepatobiliary phase. Severe intrahepatic BD stricture and dilatation in S2–S3 lobe is observed (A). Eight years later, the patient developed a severe atrophy of S2–S3 (white arrow) and hypertrophy of S1 (black arrow) (B).
Figure 5
Figure 5
Biliary wall thickening and mural enhancement of the intrahepatic biliary ducts after contrast. T1-weighted sequences after Gd-EOB-DTPA injection.
Figure 6
Figure 6
Possible gallbladder findings in PSC. Gallbladder mass observed in T2 weighted (A) and T1 weighted after contrast images (B). MRCP showing an enlarged gallbladder in PSC (C).
Figure 7
Figure 7
Intrahepatic pericholecystic cholangiocarcinoma. T1-weighted contrast-enhanced portal phase (A), T2-weighted sequence showing hyperintensity due to increased cellularity (B) and T1-weighted contrast-enhanced (Gd-EOB-DTPA) hepatospecific phase showing hypointensity due to a lack of hepatocellular contrast uptake (C).
Figure 8
Figure 8
A medium-size intrahepatic bile duct with concentric periductal fibrosis (i.e., onion skin fibrosis) in a PSC biopsy (hematoxylin–eosin; original magnification 20×).
See this image and copyright information in PMC

Similar articles

See all similar articles

References

    1. Karlsen T.H., Folseraas T., Thorburn D., Vesterhus M. Primary sclerosing cholangitis—A comprehensive review. J. Hepatol. 2017;67:1298–1323. doi: 10.1016/j.jhep.2017.07.022. - DOI - PubMed
    1. Aadland E., Schrumpf E., Fausa O., Elgjo K., Heilo A., Aakhus T., Gjone E. Primary sclerosing cholangitis: A long-term follow-up study. Scand. J. Gastroenterol. 1987;22:655–664. doi: 10.3109/00365528709011139. - DOI - PubMed
    1. Weismüller T.J., Trivedi P.J., Bergquist A., Imam M., Lenzen H., Ponsioen C.Y., Holm K., Gotthardt D., Färkkilä M.A., Marschall H.-U., et al. Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate with Course of Primary Sclerosing Cholangitis. Gastroenterology. 2017;152:1975–1984.e8. doi: 10.1053/j.gastro.2017.02.038. - DOI - PMC - PubMed
    1. Boonstra K., Weersma R.K., van Erpecum K.J., Rauws E.A., Spanier B.W.M., Poen A.C., van Nieuwkerk K.M., Drenth J.P., Witteman B.J., Tuynman H.A., et al. Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis. Hepatology. 2013;58:2045–2055. doi: 10.1002/hep.26565. - DOI - PubMed
    1. Terziroli Beretta-Piccoli B., Vergani D., Mieli-Vergani G. Autoimmune sclerosing cholangitis: Evidence and open questions. J. Autoimmun. 2018;95:15–25. doi: 10.1016/j.jaut.2018.10.008. - DOI - PubMed

MeSH terms

Grants and funding

This research received no external funding.

LinkOut - more resources

-