Primary sclerosing cholangitis: A review and update

Tabibian, James H.; Bowlus, Christopher L.

doi:10.1016/j.livres.2017.12.002

Cited by 44 publications

(54 citation statements)

References 192 publications

(212 reference statements)

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“…The decline of PBC as a reason for listing is likely influenced by the rise of ursodeoxycholic acid as a validated and effective treatment since 1994 [12]. Interestingly, the effect is not the same for PSC, where the use of ursodeoxycholic acid has also garnered interest, although without the same level of confidence in the benefits [13]. This decline is also probably influencing the decrease in listing for autoimmune liver disease overall, as seen between 1996-2007 and 2008-2016.…”

Section: Discussionmentioning

confidence: 99%

Mortality on the UNOS Waitlist for Patients with Autoimmune Liver Disease

Suri

Danford

Patwardhan

et al. 2020

JCM

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Background: Outcomes on the liver transplant waitlist can vary by etiology. Our aim is to investigate differences in waitlist mortality of autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) using the United Network for Organ Sharing (UNOS) database. Methods: We identified patients who were listed for liver transplantation from 1987 to 2016 with a primary diagnosis of AIH, PBC, or PSC. We excluded patients with overlap syndromes, acute hepatic necrosis, missing data, and those who were children. The primary outcome was death or removal from the waitlist due to clinical deterioration. We compared waitlist survival using competing risk analysis. Results: Between 1987 and 2016, there were 7412 patients listed for liver transplant due to AIH, 8119 for PBC, and 10,901 for PSC. Patients with AIH were younger, more likely to be diabetic, and had higher listing model for end-stage liver disease (MELD) scores compared to PBC and PSC patients. Patients with PBC and AIH were more likely to be removed from the waitlist due to death or clinical deterioration. On competing risk analysis, AIH patients had a similar risk of being removed from the waitlist compared to those with PBC (subdistribution hazard ratio (SHR) 0.94, 95% CI 0.85–1.03) and higher risk of removal compared to those with PSC (SHR 0.8, 95% CI 0.72 to 0.89). Conclusion: Autoimmune hepatitis carries a similar risk of waitlist removal to PBC and a higher risk than PSC. The etiology of this disparity is not entirely clear and deserves further investigation.

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Section: Discussionmentioning

confidence: 99%

Mortality on the UNOS Waitlist for Patients with Autoimmune Liver Disease

Suri

Danford

Patwardhan

et al. 2020

JCM

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“…PSC and Primary Biliary Cholangitis (PBC) are rare cholestatic liver diseases that affect the biliary system. PSC is an idiopathic disease with cholestasis, inflammation and eventual fibrosis resulting from strictures of intra-and extrahepatic bile ducts (33). PSC is one of the most common causes for liver transplantation (LT) (33).…”

Section: Dysregulation Of Bile Acids In Chronic Liver Diseases Psc Anmentioning

confidence: 99%

“…PSC is an idiopathic disease with cholestasis, inflammation and eventual fibrosis resulting from strictures of intra-and extrahepatic bile ducts (33). PSC is one of the most common causes for liver transplantation (LT) (33). Due to its heterogenous and spontaneous progression, effective medical therapies have not yet been developed (33).…”

Section: Dysregulation Of Bile Acids In Chronic Liver Diseases Psc Anmentioning

confidence: 99%

Bile Acid Receptor Therapeutics Effects on Chronic Liver Diseases

et al. 2020

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In the past ten years, our understanding of the importance of bile acids has expanded from fat absorption and glucose/lipid/energy homeostasis into potential therapeutic targets for amelioration of chronic cholestatic liver diseases. The discovery of important bile acid signaling mechanisms, as well as their role in metabolism, has increased the interest in bile acid/bile acid receptor research development. Bile acid levels and speciation are dysregulated during liver injury/damage resulting in cytotoxicity, inflammation, and fibrosis. An increasing focus to target bile acid receptors, responsible for bile acid synthesis and circulation, such as Farnesoid X receptor and apical sodium-dependent bile acid transporter to reduce bile acid synthesis have resulted in clinical trials for treatment of previously untreatable chronic liver diseases such as non-alcoholic steatohepatitis and primary sclerosing cholangitis. This review focuses on current bile acid receptor mediators and their effects on parenchymal and non-parenchymal cells. Attention will also be brought to the gut/liver axis during chronic liver damage and its treatment with bile acid receptor modulators. Overall, these studies lend evidence to the importance of bile acids and their receptors on liver disease establishment and progression.

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“…As PSC progresses, the bile ducts will be destroyed, and the patient will develop end-stage liver disease. Additionally, PSC is also associated with an increased risk for malignancies, hepatobiliary cancer [22] as well as extrahepatic malignancies such as colorectal cancer [23,24]. Lacking effective treatment options, liver transplantation is often the only treatment [25].…”

Section: Primary Sclerosing Cholangitismentioning

confidence: 99%

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

Karlsson

2020

Linköping University Medical Dissertations

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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.

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Primary sclerosing cholangitis: A review and update

Cited by 44 publications

References 192 publications

Mortality on the UNOS Waitlist for Patients with Autoimmune Liver Disease

Mortality on the UNOS Waitlist for Patients with Autoimmune Liver Disease

Bile Acid Receptor Therapeutics Effects on Chronic Liver Diseases

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

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