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https://www.readbyqxmd.com/read/29023915/a-randomized-trial-of-obeticholic-acid-monotherapy-in-patients-with-primary-biliary-cholangitis
#1
Kris V Kowdley, Velimir Luketic, Roger Chapman, Gideon M Hirschfield, Raoul Poupon, Christoph Schramm, Catherine Vincent, Christian Rust, Albert Parés, Andrew Mason, David Shapiro, Luciano Adorini, Cathi Sciacca, Tessa Beecher-Jones, Olaf Böhm, Richard Pencek, David Jones
BACKGROUND: Obeticholic acid (OCA), a potent farnesoid X receptor (FXR) agonist, was studied as monotherapy in an international, randomized, double-blind, placebo-controlled Phase 2 study in patients with primary biliary cholangitis (PBC) who were then reported for up to 6 years. The goals of the study were to assess the benefit of OCA in the absence of UDCA, which is relevant for patients who are intolerant of UDCA and at higher risk of disease progression. METHODS: Patients were randomized and dosed with placebo (n=23), OCA 10-mg (n=20), or OCA 50-mg (n=16) given as monotherapy once daily for 3 months (1 randomized patient withdrew prior to dosing)...
October 10, 2017: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/28844936/toronto-hcc-risk-index-a-validated-scoring-system-to-predict-10-year-risk-of-hcc-in-patients-with-cirrhosis
#2
Suraj A Sharma, Matthew Kowgier, Bettina E Hansen, Willem Pieter Brouwer, Raoel Maan, David Wong, Hemant Shah, Korosh Khalili, Colina Yim, E Jenny Heathcote, Harry L A Janssen, Morris Sherman, Gideon M Hirschfield, Jordan J Feld
BACKGROUND & AIMS: Current guidelines recommend biannual surveillance for hepatocellular carcinoma (HCC) in all patients with cirrhosis, regardless of etiology. However, HCC incidence is not well established for many causes of cirrhosis. We aimed to assess the disease-specific incidence of HCC in a large cohort of patients with cirrhosis and to develop a scoring system to predict HCC risk. METHODS: A derivation cohort of patients with cirrhosis diagnosed by biopsy or non-invasive measures was identified through retrospective chart review...
August 24, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28839868/evolving-strategies-to-reduce-colectomy-rates-in-primary-sclerosing-cholangitis-inflammatory-bowel-disease-clinical-remission-of-corticosteroid-refractory-colitis-post-liver-transplant-with-vedolizumab
#3
Saqib Mumtaz, Jason Goh, Gideon M Hirschfield, James Ferguson, Sheldon C Cooper
Primary sclerosing cholangitis (PSC) is associated with inflammatory bowel disease (IBD) in approximately 70% of patients, with ulcerative colitis (UC) being the most common subtype of IBD identified. There is a paucity of data on the optimum management strategy for IBD flares in the post-liver transplant patient, particularly when refractory to conventional treatments. Vedolizumab is a novel gut-specific monoclonal antibody, which has recently been approved for use by National Institute for Health and Care Excellence for moderate-to-severe UC...
October 2016: Frontline Gastroenterology
https://www.readbyqxmd.com/read/28839864/autoimmune-liver-disease-evaluating-overlapping-and-cross-over-presentations-a-case-based-discussion
#4
Margaret Corrigan, Gideon M Hirschfield
The three classic autoimmune liver diseases are recognised based on identifying varying clinical, laboratory, histological and radiological features that collectively classify patients. In the absence of defined aetiological factors, it is recognised that disease spectrum is broad, and, in this context, it is not infrequent for disease boundaries to be blurred, leading to overlapping features that may be present at the time of diagnosis or may appear later in the course of disease. Given the absence of accepted diagnostic criteria for overlap/cross-over syndromes, alongside weak data for intervention, it is recommended that a multidisciplinary, patient-specific approach be used to establish individual treatment pathways...
October 2016: Frontline Gastroenterology
https://www.readbyqxmd.com/read/28818518/seladelpar-mbx-8025-a-selective-ppar-%C3%AE-agonist-in-patients-with-primary-biliary-cholangitis-with-an-inadequate-response-to-ursodeoxycholic-acid-a-double-blind-randomised-placebo-controlled-phase-2-proof-of-concept-study
#5
David Jones, Pol F Boudes, Mark G Swain, Christopher L Bowlus, Michael R Galambos, Bruce R Bacon, Yvonne Doerffel, Norman Gitlin, Stuart C Gordon, Joseph A Odin, David Sheridan, Markus-Alexander Wörns, Virginia Clark, Linsey Corless, Heinz Hartmann, Mark E Jonas, Andreas E Kremer, George F Mells, Peter Buggisch, Bradley L Freilich, Cynthia Levy, John M Vierling, David E Bernstein, Marek Hartleb, Ewa Janczewska, Fedja Rochling, Hemant Shah, Mitchell L Shiffman, John H Smith, Yun-Jung Choi, Alexandra Steinberg, Monika Varga, Harinder Chera, Robert Martin, Charles A McWherter, Gideon M Hirschfield
BACKGROUND: Many patients with primary biliary cholangitis have an inadequate response to first-line therapy with ursodeoxycholic acid. Seladelpar is a potent, selective agonist for the peroxisome proliferator-activated receptor-delta (PPAR-δ), which is implicated in bile acid homoeostasis. This first-in-class study evaluated the anti-cholestatic effects and safety of seladelpar in patients with an inadequate response to ursodeoxycholic acid. METHODS: The study was a 12-week, double-blind, placebo-controlled, phase 2 trial of patients with alkaline phosphatase of at least 1·67 times the upper limit of normal (ULN) despite treatment with ursodeoxycholic acid...
August 14, 2017: Lancet. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28794437/genetic-variation-at-the-cd28-locus-and-its-impact-on-expansion-of-pro-inflammatory-cd28-negative-t-cells-in-healthy-individuals
#6
Evaggelia Liaskou, Louisa Jeffery, Dimitrios Chanouzas, Blagoje Soskic, Michael F Seldin, Lorraine Harper, David Sansom, Gideon M Hirschfield
The CD28 locus is associated with susceptibility to a variety of autoimmune and immune-mediated inflammatory diseases including primary sclerosing cholangitis (PSC). Previously, we linked the CD28 pathway in PSC disease pathology and found that vitamin D could maintain CD28 expression. Here, we assessed whether the PSC-associated CD28 risk variant A (rs7426056) affects CD28 expression and T cell function in healthy individuals (n = 14 AA, n = 14 AG, n = 14 GG). Homozygotes for the PSC disease risk allele (AA) showed significantly lower CD28 mRNA expression ex-vivo than either GG or AG (p < 0...
August 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28701337/should-we-screen-for-cirrhosis
#7
Mark Hudson, Nick Sheron, Ian A Rowe, Gideon M Hirschfield
No abstract text is available yet for this article.
July 12, 2017: BMJ: British Medical Journal
https://www.readbyqxmd.com/read/28692793/rare-x-chromosome-abnormalities-in-systemic-lupus-erythematosus-and-sj%C3%A3-gren-s-syndrome
#8
Rohan Sharma, Valerie M Harris, Joshua Cavett, Biji T Kurien, Ke Liu, Kristi A Koelsch, Anum Fayaaz, Kaustubh S Chaudhari, Lida Radfar, David Lewis, Donald U Stone, C Erick Kaufman, Shibo Li, Barbara Segal, Daniel J Wallace, Michael H Weisman, Swamy Venuturupalli, Jennifer A Kelly, Bernardo Pons-Estel, Roland Jonsson, Xianglan Lu, Jacques-Eric Gottenberg, Juan-Manuel Anaya, Deborah S Cunninghame-Graham, Andrew J W Huang, Michael T Brennan, Pamela Hughes, Ilias Alevizos, Corinne Miceli-Richard, Edward C Keystone, Vivian P Bykerk, Gideon Hirschfield, Gunnel Nordmark, Sara Magnusson Bucher, Per Eriksson, Roald Omdal, Nelson L Rhodus, Maureen Rischmueller, Michael Rohrer, Marie Wahren-Herlenius, Torsten Witte, Marta Alarcón-Riquelme, Xavier Mariette, Christopher J Lessard, John B Harley, Wan-Fai Ng, Astrid Rasmussen, Kathy L Sivils, R Hal Scofield
OBJECTIVE: Sjögren's syndrome (SS) and systemic lupus erythematosus (SLE) are related by clinical and serologic manifestations as well as genetic risks. Both diseases are more commonly found in women than in men, at a ratio of ~10 to 1. Common X chromosome aneuploidies, 47,XXY and 47,XXX, are enriched among men and women, respectively, in either disease, suggesting a dose effect on the X chromosome. METHODS: We examined cohorts of SS and SLE patients by constructing intensity plots of X chromosome single-nucleotide polymorphism alleles, along with determining the karyotype of selected patients...
July 10, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28690174/clinical-outcomes-of-donation-after-circulatory-death-liver-transplantation-in-primary-sclerosing-cholangitis
#9
Palak J Trivedi, Irene Scalera, Emma Slaney, Richard W Laing, Bridget Gunson, Gideon M Hirschfield, Andrea Schlegel, James Ferguson, Paolo Muiesan
BACKGROUND & AIM: Primary sclerosing cholangitis (PSC) is a progressive fibro-inflammatory cholangiopathy for which liver transplantation is the only life-extending intervention. These patients may benefit from accepting liver donation after circulatory death (DCD), however their subsequent outcome is unknown. The aim of this study was to determine the clinical impact of using DCD liver grafts in patients specifically undergoing transplantation for PSC. METHODS: Clinical outcomes were prospectively evaluated in PSC patients undergoing transplantation from 2006 to 2016 stratified by donor type (DCD, n=35 vs...
July 8, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28674140/investigating-the-safety-and-activity-of-the-use-of-btt1023-timolumab-in-the-treatment-of-patients-with-primary-sclerosing-cholangitis-buteo-a-single-arm-two-stage-open-label-multi-centre-phase-ii-clinical-trial-protocol
#10
Katherine Arndtz, Margaret Corrigan, Anna Rowe, Amanda Kirkham, Darren Barton, Richard P Fox, Laura Llewellyn, Amrita Athwal, Manpreet Wilkhu, Yung-Yi Chen, Chris Weston, Amisha Desai, David H Adams, Gideon M Hirschfield
INTRODUCTION: Primary sclerosing cholangitis (PSC) is a progressive inflammatory liver disease characterised by relentless liver fibrosis and a high unmet need for new therapies. Preventing fibrosis represents an important area of interest in the development of vital new drugs. Vascular adhesion protein-1 (VAP-1) drives inflammation in liver disease, and provision of an antibody against VAP-1 blunts fibrosis in murine models of liver injury. METHODS AND ANALYSIS: BUTEO is a single-arm, two-stage, open-label, multi-centre, phase II clinical trial...
July 3, 2017: BMJ Open
https://www.readbyqxmd.com/read/28529147/norursodeoxycholic-acid-improves-cholestasis-in-primary-sclerosing-cholangitis
#11
Peter Fickert, Gideon M Hirschfield, Gerald Denk, Hanns-Ulrich Marschall, Istvan Altorjay, Martti Färkkilä, Christoph Schramm, Ulrich Spengler, Roger Chapman, Annika Bergquist, Erik Schrumpf, Frederik Nevens, Palak Trivedi, Florian P Reiter, Istvan Tornai, Emina Halilbasic, Roland Greinwald, Markus Pröls, Michael P Manns, Michael Trauner
BACKGROUND & AIM: Primary sclerosing cholangitis (PSC) represents a devastating bile duct disease, currently lacking effective medical therapy. 24-norursodeoxycholic acid (norUDCA) is a side chain-shortened C23 homologue of UDCA and has shown potent anti-cholestatic, anti-inflammatory and anti-fibrotic properties in a preclinical PSC mouse model. A randomized controlled trial, including 38 centers from 12 European countries, evaluated the safety and efficacy of three doses of oral norUDCA (500mg/d, 1,000mg/d or 1,500mg/d) compared with placebo in patients with PSC...
September 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28468019/preface
#12
Gideon Hirschfield
No abstract text is available yet for this article.
2017: Digestive Diseases
https://www.readbyqxmd.com/read/28428344/vascular-adhesion-protein-1-is-elevated-in-primary-sclerosing-cholangitis-is-predictive-of-clinical-outcome-and-facilitates-recruitment-of-gut-tropic-lymphocytes-to-liver-in-a-substrate-dependent-manner
#13
Palak J Trivedi, Joseph Tickle, Mette Nåmdal Vesterhus, Peter J Eddowes, Tony Bruns, Jani Vainio, Richard Parker, David Smith, Evaggelia Liaskou, Liv Wenche Thorbjørnsen, Gideon M Hirschfield, Kaisa Auvinen, Stefan G Hubscher, Marko Salmi, David H Adams, Chris J Weston
OBJECTIVE: Primary sclerosing cholangitis (PSC) is the classical hepatobiliary manifestation of IBD. This clinical association is linked pathologically to the recruitment of mucosal T cells to the liver, via vascular adhesion protein (VAP)-1-dependent enzyme activity. Our aim was to examine the expression, function and enzymatic activation of the ectoenzyme VAP-1 in patients with PSC. DESIGN: We examined VAP-1 expression in patients with PSC, correlated levels with clinical characteristics and determined the functional consequences of enzyme activation by specific enzyme substrates on hepatic endothelium...
April 20, 2017: Gut
https://www.readbyqxmd.com/read/28427765/easl-clinical-practice-guidelines-the-diagnosis-and-management-of-patients-with-primary-biliary-cholangitis
#14
(no author information available yet)
Primary biliary cholangitis (PBC) is a chronic inflammatory autoimmune cholestatic liver disease, which when untreated will culminate in end-stage biliary cirrhosis. 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 and risk stratification is important to ensure all patients receive a personalised approach to their care. The goals of treatment and management are the prevention of end-stage liver disease, and the amelioration of associated symptoms...
April 18, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/28420030/role-of-endoscopy-in-primary-sclerosing-cholangitis-european-society-of-gastrointestinal-endoscopy-esge-and-european-association-for-the-study-of-the-liver-easl-clinical-guideline
#15
Lars Aabakken, Tom H Karlsen, Jörg Albert, Marianna Arvanitakis, Olivier Chazouilleres, Jean-Marc Dumonceau, Martti Färkkilä, Peter Fickert, Gideon M Hirschfield, Andrea Laghi, Marco Marzioni, Michael Fernandez, Stephen P Pereira, Jürgen Pohl, Jan-Werner Poley, Cyriel Y Ponsioen, Christoph Schramm, Fredrik Swahn, Andrea Tringali, Cesare Hassan
1 ESGE/EASL recommend that, as the primary diagnostic modality for PSC, magnetic resonance cholangiography (MRC) should be preferred over endoscopic retrograde cholangiopancreatography (ERCP).Moderate quality evidence, strong recommendation. 2 ESGE/EASL suggest that ERCP can be considered if MRC plus liver biopsy is equivocal or contraindicated in patients with persisting clinical suspicion of PSC. The risks of ERCP have to be weighed against the potential benefit with regard to surveillance and treatment recommendations...
April 18, 2017: Endoscopy
https://www.readbyqxmd.com/read/28349484/in-vitro-and-ex-vivo-models-to-study-t-cell-migration-through-the-human-liver-parenchyma
#16
Benjamin G Wiggins, Konstantinos Aliazis, Scott P Davies, Gideon Hirschfield, Patricia F Lalor, Gary Reynolds, Zania Stamataki
The liver is the largest internal organ and filters around 3 pints of blood per minute. This continuous flux of blood should not be confused with rapid egress of lymphocytes through the liver; this organ has intricate corridors of specialized sinusoidal spaces, ensuring that immune cells decelerate to shear flow rates, and providing ample opportunities to interact with parenchymal cells. Migration has been intricately linked to T cell function; it is therefore important to study liver T cell biology into context within the liver microenvironment...
2017: Methods in Molecular Biology
https://www.readbyqxmd.com/read/28274849/patient-age-sex-and-inflammatory-bowel-disease-phenotype-associate-with-course-of-primary-sclerosing-cholangitis
#17
MULTICENTER STUDY
Tobias J Weismüller, Palak J Trivedi, Annika Bergquist, Mohamad Imam, Henrike Lenzen, Cyriel Y Ponsioen, Kristian Holm, Daniel Gotthardt, Martti A Färkkilä, Hanns-Ulrich Marschall, Douglas Thorburn, Rinse K Weersma, Johan Fevery, Tobias Mueller, Olivier Chazouillères, Kornelius Schulze, Konstantinos N Lazaridis, Sven Almer, Stephen P Pereira, Cynthia Levy, Andrew Mason, Sigrid Naess, Christopher L Bowlus, Annarosa Floreani, Emina Halilbasic, Kidist K Yimam, Piotr Milkiewicz, Ulrich Beuers, Dep K Huynh, Albert Pares, Christine N Manser, George N Dalekos, Bertus Eksteen, Pietro Invernizzi, Christoph P Berg, Gabi I Kirchner, Christoph Sarrazin, Vincent Zimmer, Luca Fabris, Felix Braun, Marco Marzioni, Brian D Juran, Karouk Said, Christian Rupp, Kalle Jokelainen, Maria Benito de Valle, Francesca Saffioti, Angela Cheung, Michael Trauner, Christoph Schramm, Roger W Chapman, Tom H Karlsen, Erik Schrumpf, Christian P Strassburg, Michael P Manns, Keith D Lindor, Gideon M Hirschfield, Bettina E Hansen, Kirsten M Boberg
BACKGROUND & AIMS: Primary sclerosing cholangitis (PSC) is an orphan hepatobiliary disorder associated with inflammatory bowel disease (IBD). We aimed to estimate the risk of disease progression based on distinct clinical phenotypes in a large international cohort of patients with PSC. METHODS: We performed a retrospective outcome analysis of patients diagnosed with PSC from 1980 through 2010 at 37 centers in Europe, North America, and Australia. For each patient, we collected data on sex, clinician-reported age at and date of PSC and IBD diagnoses, phenotypes of IBD and PSC, and date and indication of IBD-related surgeries...
June 2017: Gastroenterology
https://www.readbyqxmd.com/read/28249258/apical-sodium-dependent-transporter-inhibitors-in-primary-biliary-cholangitis-and-primary-sclerosing-cholangitis
#18
REVIEW
Vinod S Hegade, David E J Jones, Gideon M Hirschfield
Bile acids (BAs) have gained mainstream attention since the discovery of their key role as signalling molecules in health and disease. The apical sodium-dependent transporter (ASBT) protein located in the terminal ileum plays an important physiological role in the enterohepatic circulation of BAs and therefore essential for the BA homeostasis. Primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC), the 2 most common cholestatic liver diseases are characterised by altered BA flow and BA composition, which contribute to disease progression and symptom (pruritus) development...
2017: Digestive Diseases
https://www.readbyqxmd.com/read/28187915/effect-of-ileal-bile-acid-transporter-inhibitor-gsk2330672-on-pruritus-in-primary-biliary-cholangitis-a-double-blind-randomised-placebo-controlled-crossover-phase-2a-study
#19
Vinod S Hegade, Stuart F W Kendrick, Robert L Dobbins, Sam R Miller, Douglas Thompson, Duncan Richards, James Storey, George E Dukes, Margaret Corrigan, Ronald P J Oude Elferink, Ulrich Beuers, Gideon M Hirschfield, David E Jones
BACKGROUND: Up to 70% of patients with primary biliary cholangitis develop pruritus (itch) during the course of their disease. Treatment of pruritus in primary biliary cholangitis is challenging and novel therapies are needed. Ursodeoxycholic acid, the standard first-line treatment for primary biliary cholangitis, is largely ineffective for pruritus. We investigated the efficacy and safety of GSK2330672, a selective inhibitor of human ileal bile acid transporter (IBAT), in patients with primary biliary cholangitis with pruritus...
March 18, 2017: Lancet
https://www.readbyqxmd.com/read/28176332/low-dose-interleukin-2-promotes-stat-5-phosphorylation-treg-survival-and-ctla-4-dependent-function-in-autoimmune-liver-diseases
#20
H C Jeffery, L E Jeffery, P Lutz, M Corrigan, G J Webb, G M Hirschfield, D H Adams, Y H Oo
CD4(+) CD25(high) CD127(low) forkhead box protein 3 (FoxP3(+) ) regulatory T cells (Treg ) are essential for the maintenance of peripheral tolerance. Impaired Treg function and an imbalance between effector and Tregs contribute to the pathogenesis of autoimmune diseases. We reported recently that the hepatic microenvironment is deficient in interleukin (IL)-2, a cytokine essential for Treg survival and function. Consequently, few liver-infiltrating Treg demonstrate signal transducer and activator of transcription-5 (STAT-5) phosphorylation...
June 2017: Clinical and Experimental Immunology
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