British Society of Gastroenterology and UK-PSC guidelines for the diagnosis and management of primary sclerosing cholangitis

Chapman, Michael H.; Thorburn, Douglas; Hirschfield, Gideon; Webster, GJ; Rushbrook, Simon; Alexander, Graeme; Collier, Jane; Dyson, Jessica; Jones, David; Patanwala, Imran; Thain, Collette; Walmsley, Martine; Pereira, Stephen P.

doi:10.1136/gutjnl-2018-317993

Cited by 193 publications

(284 citation statements)

References 288 publications

(237 reference statements)

Supporting

Mentioning

250

Contrasting

Unclassified

Order By: Relevance

“…Other invasive scores that are useful in selected cases are APRI (aspartate aminotransferase to platelet ratio index), Fib, Enhanced Liver Fibrosis ELF score, liver stiffness (noninvasive tests), Amsterdam score (based on intrahepatic and extrahepatic changes on cholangiography), Ludwigand Batt's, Nakanuma, and Ishak scores (histology scores) [2]. Most deaths are attributable to cholangiocarcinoma (58%), liver failure (30%), and variceal bleeding (9%) [5].…”

Section: Natural History and Prognostic Modelsmentioning

confidence: 99%

“…Bilirubin can be normal or can fluctuate in time, and higher values can be a marker of poor prognosis. Low albumin can show decompensated disease with hepatic synthetic dysfunction, malnutrition, or active IBD [2,5].…”

Section: Laboratory Testsmentioning

confidence: 99%

“…Transient elastography and serological tests for assessment of liver fibrosis are more commonly used in staging liver fibrosis and predict prognosis in patients with PSC. Magnetic elastography is gaining more and more importance as a noninvasive test for liver fibrosis [5].…”

Section: Noninvasive Tests For Assessing Liver Fibrosismentioning

confidence: 99%

“…In most of the cases, the diagnosis is now established through magnetic resonance cholangiography (MRCP) showing multifocal strictures and saccular dilatations of the bile ducts ("beaded" appearance) [4]. Recently, diagnosis of PSC is made by cholestatic liver biochemistry with typical cholangiographic aspect in the absence of other causes of secondary sclerosing cholangitis [5].…”

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

An Overview on Primary Sclerosing Cholangitis

Vlăduţ

Ciocîrlan

Bilous

et al. 2020

JCM

View full text Add to dashboard Cite

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.

show abstract

Section: Natural History and Prognostic Modelsmentioning

confidence: 99%

Section: Laboratory Testsmentioning

confidence: 99%

Section: Noninvasive Tests For Assessing Liver Fibrosismentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 2 more Smart Citations

An Overview on Primary Sclerosing Cholangitis

Vlăduţ

Ciocîrlan

Bilous

et al. 2020

JCM

View full text Add to dashboard Cite

show abstract

“…Second, the macroscopic findings on MRCP do not seem to correlate well with liver function. This could be interpreted as indicating that MRCP should not be used to assess parenchymal disease progression, despite being useful for diagnosing PSC and screening for tumors [155]. It could be that the parenchymal progression is mainly driven by microscopic changes, e.g.…”

Section: Association Between Gadoxetate-enhanced Mri and Mrcpmentioning

confidence: 99%

Non-Invasive Characterization of Liver Disease : By Multimodal Quantitative Magnetic Resonance

Karlsson

2020

Linköping University Medical Dissertations

View full text Add to dashboard Cite

There is a large and unmet need for diagnostic tool that can be used to characterize chronic liver diseases (CLD). In the earlier stages of CLD, much of the diagnostics involves performing biopsies, which are evaluated by a histopathologist for the presence of e.g. fat, iron, inflammation, and fibrosis. Performing biopsies, however, have two downsides: i) biopsies are invasive and carries a small but non-negligible risk for serious complications, ii) biopsies only represents a tiny portion of the liver and are thus prone to sampling error. Moreover, in the later stages of CLD, when the disease has progressed far enough, the ability of the liver to perform its basic function will be compromised. In this stage, there is a need for better methods for accurately measuring liver function. Additionally, measures of liver function can also be used when developing new drugs, as biomarkers for drug-induced liver injury (DILI), which is a serious drug-safety issue. Magnetic resonance imaging (MRI) is a non-invasive medical imaging modality, which have shown much promise with regards to characterizing liver disease in all of the above-mentioned aspects. The aim of this PhD project was to develop and validate MR-based methods that can be used to non-invasively characterize liver disease. Paper I investigated if R2* mapping, a MR-method for measuring liver iron content, can be confounded by liver fat. The results show fat does affect R2*. The conclusion was therefore that fat must be taken into account when measuring small amounts of liver iron, as a small increase in R2* could be due to either small amounts of iron or large amounts of fat. Paper II examined whether T1 mapping, which is another MR-method, can be used for staging liver fibrosis. The results of previous research have been mixed; some studies have been very promising, whereas other studies have been less promising. Unfortunately, the results in Paper II belongs to the less promising studies. Paper III focused on measuring liver function by dynamic contrast-enhanced MRI (DCE-MRI) using a liver specific contrast agent, which is taken up the hepatocytes and excreted to the bile. The purpose of the paper was to extend and validate a method for estimating uptake and efflux rates of the contrast agent. The method had previously only been applied in health volunteers. Paper II showed that the method can be applied to CLD patients and that the uptake of the contrast agent is lower in patients with advanced fibrosis.

show abstract