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Review
. 2020 Mar 11;9(3):754.
doi: 10.3390/jcm9030754.

An Overview on Primary Sclerosing Cholangitis

Cătălina Vlăduţ  1   2 , Mihai Ciocîrlan  1   2 , Dana Bilous  2 , Vasile Șandru  3 , Mădălina Stan-Ilie  2   3 , Nikola Panic  4 , Gabriel Becheanu  5 , Mariana Jinga  2   6 , Raluca S Costache  2   6 , Daniel O Costache  7 , Mircea Diculescu  2   8
Affiliations
Review

An Overview on Primary Sclerosing Cholangitis

Cătălina Vlăduţ et al. J Clin Med. .

Abstract

Primary sclerosing cholangitis is a progressive liver disease characterized by chronic inflammation leading to liver fibrosis and cirrhosis. Even though the exact pathogenesis is still unclear, a combination of autoimmune, environmental, and ischemic factors could explain certain aspects of the disease. The most important diagnostic step is cholangiography, which can be obtained either by endoscopic retrograde cholangiopancreatography (ERCP), magnetic resonance cholangiography (MRCP as the gold standard), or percutaneous transhepatic cholangiography. It shows multifocal short biliary duct strictures leading to the "beaded" aspect. Cholangiocarcinoma and colorectal adenocarcinoma are the most feared complications in patients with Primary sclerosing cholangitis (PSC). Continuous screening consists of annual clinical, biochemical, and ultrasound assessments in asymptomatic patients and annual colonoscopy in patients with PSC and inflammatory bowel disease. In newly diagnosed patients with PSC, colonoscopy is mandatory and, if negative, then, a repeat colonoscopy should be performed in 3-5 years. The lack of efficient curative medical treatment makes invasive treatments such as liver transplant and endoscopy the mainstream for managing PSC and its complications. Until now, even though only ursodeoxycholic acid has shown a moderate clinical, biochemical, and even histological improvement, it has no significant influence on the risk of cholangiocarcinoma, liver transplant need, or death risk and it is no longer recommended in treating early PSC. Further studies are in progress to establish the effect of molecular-targeted therapies in PSC.

Keywords: cholangiography; choleangiectasias; liver transplant; primary sclerosing cholangitis.

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Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Factors involved in etiopathogenesis of Primary sclerosing cholangitis (PSC) [1,6].
Figure 2
Figure 2
Cholangiography of a patient with PSC from our clinical practice: (a) magnetic resonance cholangiography (MRCP) images; (b) endoscopic retrograde cholangiopancreatography (ERCP) images.
Figure 3
Figure 3
Portal space with periductal fibrosis and moderate inflammation hematoxylin eosin (HE) 200×.
Figure 4
Figure 4
Periductal fibrosis—Van Gieson coloration 400×.
See this image and copyright information in PMC

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