Cholestatic Liver Injury: Care of Patients With Primary Biliary Cholangitis or Primary Sclerosing Cholangitis

2016 ◽  
Vol 27 (4) ◽  
pp. 441-452 ◽  
Author(s):  
Laurie Larson ◽  
Michelle James ◽  
Andrea Gossard
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Disease Recurrence ◽  
Outpatient Setting ◽  
Primary Biliary Cholangitis ◽  
Malignant Neoplasms ◽  
Cholestatic Liver Disease ◽  
Gallbladder Polyps ◽  
Increased Risk ◽  
Common Causes

The most common causes of chronic cholestatic liver disease are primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). Both disease processes are characterized by a destruction of intrahepatic and/or extrahepatic biliary ducts. The etiology is not entirely clear; however, there is an underlying autoimmune component contributing to both disease processes. Although PBC and PSC are often diagnosed and managed in the outpatient setting, in some instances, a patient may have jaundice, fatigue, and pruritus requiring evaluation and determination of the cholestatic cause. Patients with PSC should be monitored for evidence of cholangiocarcinoma, colon cancer, and gallbladder polyps as they are at an increased risk of malignant neoplasms. Liver transplant has the potential for improving quality of life, although disease recurrence is a risk.

scholarly journals Novel Insights of Primary Sclerosing Cholangitis and Primary Biliary Cholangitis

2020 ◽  
Vol 25 (2) ◽  
pp. 107-117
Author(s):  
Dong-Won Ahn
Keyword(s):  
Liver Transplantation ◽  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Liver Diseases ◽  
Sclerosing Cholangitis ◽  
Acute Cholangitis ◽  
Magnetic Resonance Cholangiopancreatography ◽  
Primary Biliary Cholangitis ◽  
Cholestatic Liver Disease ◽  
Biliary Cirrhosis

Primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC) are immune-mediated chronic liver diseases. PSC is a rare disorder characterized by multi-focal bile duct strictures and progressive liver diseases, in which liver transplantation is required ultimately in most patients. Imaging studies such as magnetic resonance cholangiopancreatography have important role in diagnosis in most cases of PSC. PSC is usually accompanied by inflammatory bowel disease and there is a high risk of cholangiocarcinoma and colorectal cancer in PSC. No medical therapies have been proven to delay progression of PSC. Endoscopic intervention for tissue diagnosis or biliary drainage is frequently required in cases of PSC with dominant stricture, acute cholangitis, or clinically suspected cholangiocarcinoma. PBC is a chronic inflammatory autoimmune cholestatic liver disease, which when untreated will culminate in endstage biliary cirrhosis requiring liver transplantation. Diagnosis is usually based on the presence of serum liver tests indicative of a cholestatic hepatitis in association with circulating antimitochondrial antibodies. Patient presentation and course can be diverse in PBC and risk stratification is important to ensure all patients receive a personalised approach to their care. Medical therapy using ursodeoxycholic acid (UDCA) or obeticholic acid (OCA) has an important role to reduce the progression to end-stage liver disease in PBC.

scholarly journals Management of primary sclerosing cholangitis and its complications: an algorithmic approach

2020 ◽  
Author(s):  
Michal Prokopič ◽  
Ulrich Beuers
Keyword(s):  
Liver Disease ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Treatment Options ◽  
Diagnostic Procedures ◽  
Disease Recurrence ◽  
Cholestatic Liver Disease ◽  
Diagnostic Challenge ◽  
Algorithmic Approach ◽  
End Stage

AbstractPrimary sclerosing cholangitis (PSC) is a rare cholestatic liver disease, characterized by multiple strictures and dilatations of the intra- and extrahepatic bile ducts, leading to progressive liver fibrosis, in 10–15% cholangiocarcinoma, and ultimately end-stage liver disease. The pathogenesis is poorly understood, but (epi-)genetic factors, mechanisms of innate and adaptive immunity, toxic effects of hydrophobic bile acids, and possibly intestinal dysbiosis appear to be involved. The strong link with inflammatory bowel disease (IBD) is associated with a markedly enhanced risk of colorectal cancer which next to cholangiocarcinoma represents the most serious diagnostic challenge in long-term PSC management. Despite extensive research, no medical treatment has been proven so far to prolong the time to liver transplantation (LTx), which remains the effective treatment in late-stage disease. Recurrence of PSC after LTx is observed in up to 20% of patients. Here, we briefly summarize actual views on PSC pathogenesis and provide an algorithmic approach to diagnostic procedures and recommendations for the management of PSC and its complications. We describe promising treatment options subject to current clinical trials.

Magnetic Resonance Imaging in Primary Sclerosing Cholangitis—Current State and Future Directions

2019 ◽  
Vol 39 (03) ◽  
pp. 369-380 ◽  
Author(s):  
Roman Zenouzi ◽  
Christopher L. Welle ◽  
Sudhakar K. Venkatesh ◽  
Christoph Schramm ◽  
John E. Eaton
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Imaging Modality ◽  
Magnetic Resonance Cholangiopancreatography ◽  
Cholestatic Liver Disease ◽  
Resonance Imaging ◽  
Look Ahead ◽  
Increased Risk

AbstractPrimary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease characterized by biliary inflammation and fibrosis leading to bile duct strictures, cirrhosis, and carries an increased risk of hepatobiliary malignancies. Magnetic resonance imaging (MRI) with magnetic resonance cholangiopancreatography (MRCP) is the imaging modality of choice in PSC. As an evolving technology, MRI has other potential applications in the care and study of those patients with PSC. In this review, the authors aim to provide a technical overview on MRI/MRCP and related technologies, summarize its contemporary use in PSC, and discuss its evolving role to predict outcomes and look ahead toward emerging MRI technologies relevant to PSC.

scholarly journals Cholangioscopy and its Primary Sclerosing Cholangitis

2020 ◽  
pp. 42-53
Author(s):  
Brian Fung ◽  
Phillip Fejleh ◽  
Sooraj Tejaswi ◽  
James Tabibian
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Biliary Tract ◽  
Sclerosing Cholangitis ◽  
Stone Formation ◽  
Cholestatic Liver Disease ◽  
Biliary Strictures ◽  
Peroral Cholangioscopy ◽  
Increased Risk ◽  
Risk Of Malignancy ◽  
Hepatobiliary Complications

Primary sclerosing cholangitis (PSC) is a cholestatic liver disease characterised by chronic inflammation and fibro-obliteration of the intrahepatic and/or extrahepatic bile ducts. It is associated with numerous hepatobiliary complications including an increased risk of malignancy (in particular, cholangiocarcinoma) and biliary tract stone formation. The evaluation of biliary strictures in patients with PSC is especially challenging, with imaging and endoscopic methods having only modest sensitivity for the diagnosis of cholangiocarcinoma, and treatment of biliary strictures poses a similarly significant clinical challenge. In recent years, peroral cholangioscopy has evolved technologically and increased in popularity as an endoscopic tool that can provide direct intraductal visualisation and facilitate therapeutic manipulation of the biliary tract. However, the indications for and effectiveness of its use in patients with PSC remain uncertain, with only a few studies performed on this small but important subset of patients. In this review, the authors discuss the available data regarding the use of peroral cholangioscopy in patients with PSC, with a focus on its use in the evaluation and management of biliary strictures and stones.

scholarly journals Recent advances in understanding bile duct remodeling and fibrosis

F1000Research ◽  
2018 ◽  
Vol 7 ◽  
pp. 1165
Author(s):  
Marinda Scrushy ◽  
April O'Brien ◽  
Shannon Glaser
Keyword(s):  
Liver Disease ◽  
Bile Duct ◽  
Liver Transplant ◽  
Biliary Atresia ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Treatment Options ◽  
Primary Biliary Cholangitis ◽  
Cholestatic Liver Disease ◽  
Age Range

Cholestatic liver disease encompasses a detrimental group of diseases that are non-discriminatory in nature. These diseases occur over every age range from infancy (biliary atresia) to geriatrics (hepatitis). They also cover both genders in the form of primary sclerosing cholangitis in men and primary biliary cholangitis in women. Oftentimes, owing to the disease progression and extensive scarring, the treatment of last resort becomes a liver transplant. In this review, we will briefly discuss and explore new avenues of understanding in the progression of cholestatic liver disease and possible therapeutic targets for intervention. The greater our understanding into the idiopathic nature of cholestatic liver disease, the better our chances of discovering treatment options to halt or reverse the progression, reducing or eliminating the need for expensive and risky transplants.

scholarly journals Changes in beta-catenin expression and activation during progression of primary sclerosing cholangitis predict disease recurrence

2022 ◽  
Vol 12 (1) ◽  
Author(s):  
Mary Ayers ◽  
Silvia Liu ◽  
Aatur D. Singhi ◽  
Karis Kosar ◽  
Pamela Cornuet ◽  
...  
Keyword(s):  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Bile Ducts ◽  
Statistical Significance ◽  
Disease Recurrence ◽  
Immunohistochemical Localization ◽  
Cholestatic Liver Disease ◽  
Beta Catenin ◽  
Biliary Injury ◽  
Pathology Reports

AbstractPrimary sclerosing cholangitis (PSC) is a rare, chronic, cholestatic liver disease characterized by progressive inflammation and fibrosis of the bile ducts. We have previously demonstrated the importance of Wnt/β-catenin signaling in mouse models of PSC. In this study, we wished to determine the clinical relevance of β-catenin localization in patient samples. In livers explanted from patients diagnosed with PSC, the majority (12/16; 75%) lacked β-catenin protein expression. Biopsies from patients post-transplant were classified as recurrent or non-recurrent based on pathology reports and then scored for β-catenin activation as a function of immunohistochemical localization. Despite lack of statistical significance, patients with recurrent primary disease (n = 11) had a greater percentage of samples with nuclear, transcriptionally active β-catenin (average 58.8%) than those with no recurrence (n = 10; 40.53%), while non-recurrence is correlated with β-catenin staining at the cell surface (average 52.63% for non-recurrent vs. 27.34% for recurrent), as determined by three different methods of analysis. β-catenin score and years-to-endpoint are both strongly associated with recurrence status (p = 0.017 and p = 0.00063, respectively). Finally, there was significant association between higher β-catenin score and increased alkaline phosphatase, a marker of biliary injury and disease progression. Thus, β-catenin expression and activation changes during the progression of PSC, and its localization may be a useful prognostic tool for predicting recurrence of this disease.

Increased risk of colorectal neoplasia in patients with primary sclerosing cholangitis and ulcerative colitis: A meta-analysis

2002 ◽  
Vol 56 (1) ◽  
pp. 48-54 ◽  
Author(s):  
Roy M. Soetikno ◽  
Otto S. Lin ◽  
Paul A. Heidenreich ◽  
Harvey S. Young ◽  
Michael O. Blackstone
Keyword(s):  
Ulcerative Colitis ◽  
Primary Sclerosing Cholangitis ◽  
Sclerosing Cholangitis ◽  
Colorectal Neoplasia ◽  
Meta Analysis ◽  
Increased Risk

scholarly journals Sclerosing cholangitis and inflammatory bowel disease: which comes first?

2021 ◽  
Vol 66 (1) ◽  
pp. 39-46
Author(s):  
A. V. Nikitin ◽  
G. V. Volynets
Keyword(s):  
Inflammatory Bowel Disease ◽  
Bowel Disease ◽  
Sclerosing Cholangitis ◽  
Clinical Symptoms ◽  
Cross Reactivity ◽  
Formation Mechanisms ◽  
Malignant Neoplasms ◽  
Increased Risk ◽  
Bowel Diseases ◽  
Inflammatory Bowel

Sclerosing cholangitis is one of the most common hepatologic extraintestinal manifestations of inflammatory bowel disease. The article discusses the phenotype of the combination of sclerosing cholangitis and inflammatory bowel disease. The authors present their theories of the etiopathogenesis of sclerosing cholangitis in patients with inflammatory bowel disease, as well as some features of the phenotype of both mixed and monogenic forms of diseases.Sclerosing cholangitis in combination with inflammatory bowel disease is commonly associated with pancolitis, but the endoscopically visualized activity of inflammatory bowel diseases is significantly lower and clinical symptoms are less pronounced. The authors have established that the patients with the combination of sclerosing cholangitis and inflammatory bowel disease are at the increased risk of developing malignant neoplasms. The formation mechanisms of a combination of inflammatory bowel disease and sclerosing cholangitis remain poorly understood, although this pathology is influenced by lymphocytic cross-reactivity, aberrant recognition of microbiotic epitopes and intestinal microbiota imbalance. New biological agents aimed at correcting the interaction between the immune system and target organs may provide new ways of treatment for sclerosing cholangitis associated with inflammatory bowel disease.

scholarly journals New approach to decrease post-ERCP adverse events in patients with primary sclerosing cholangitis

2017 ◽  
Vol 05 (08) ◽  
pp. E710-E717 ◽  
Author(s):  
Udayakumar Navaneethan ◽  
Dennisdhilak Lourdusamy ◽  
Norma Gutierrez ◽  
Xiang Zhu ◽  
John Vargo ◽  
...  
Keyword(s):  
Adverse Events ◽  
Primary Sclerosing Cholangitis ◽  
Stent Placement ◽  
Sclerosing Cholangitis ◽  
Balloon Dilation ◽  
Bivariate Analysis ◽  
Contrast Injection ◽  
Group I ◽  
Increased Risk ◽  
Group Ii

Abstract Background and study aims Endoscopic retrograde cholangiopancreatography (ERCP) is often performed in patients with primary sclerosing cholangitis (PSC). Our aim was to validate a treatment approach with the objective of decreasing ERCP related adverse events (AEs). Patients and methods All patients who had undergone ERCP for PSC during the period from 2002 – 2012 were identified (group I). This group had traditional ERCP (no bile aspiration prior to contrast injection with balloon dilation and stent placement for treatment of dominant strictures). To decrease ERCP-related AEs, we changed the ERCP approach in which bile aspiration was performed prior to contrast injection and balloon dilation alone was performed for treatment of dominant strictures. This was tested prospectively in all patients undergoing ERCP for PSC from 2012 – 2014 (group II). Results The risk of overall AEs and cholangitis was relatively less in group II compared with group I [(2.1 % vs. 10.3 %; P = .38) and (0 % vs. 4.4 %; P = .68)]. On bivariate analysis, change in ERCP approach was associated with decreased risk of post-procedure cholangitis (0 % vs. 10.2 %, P = .03) and overall AE (0 % vs. 18.6 %, P = .03). There were no AEs in 22/46 patients in group II who had bile aspiration with balloon dilation. On multivariate analysis, only biliary stent placement was associated with increased risk of AEs (OR 4.10 (1.32 – 12.71); P = .02) and cholangitis (OR 5.43, 1.38 – 21.38; P = .02) respectively. Conclusion Biliary aspiration and avoidance of stenting approach after dilation of strictures during ERCP in PSC patients appears to be associated with decreased risk of cholangitis and overall AEs. Future prospective randomized controlled trials are needed to validate our observation.

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