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JOURNAL ARTICLE
Laura Martinez-Gili, Alexandros Pechlivanis, Julie A K McDonald, Sofina Begum, Jonathan Badrock, Jessica K Dyson, Rebecca Jones, Gideon Hirschfield, Stephen D Ryder, Richard Sandford, Simon Rushbrook, Douglas Thorburn, Simon D Taylor-Robinson, Mary M E Crossey, Julian R Marchesi, George Mells, Elaine Holmes, David Jones
Primary biliary cholangitis (PBC) is a chronic cholestatic liver disease with ursodeoxycholic acid (UDCA) as first-line treatment. Poor response to UDCA is associated with a higher risk of progressing to cirrhosis, but the underlying mechanisms are unclear. UDCA modulates the composition of primary and bacterial-derived bile acids (BAs). We characterized the phenotypic response to UDCA based on BA and bacterial profiles of PBC patients treated with UDCA. Patients from the UK-PBC cohort ( n = 419) treated with UDCA for a minimum of 12-months were assessed using the Barcelona dynamic response criteria...
2023: Gut Microbes
#22
JOURNAL ARTICLE
Christopher L Bowlus, Bertus Eksteen, Angela C Cheung, Douglas Thorburn, Cynthia A Moylan, Paul J Pockros, Lisa M Forman, Alejandro Dorenbaum, Gideon M Hirschfield, Ciara Kennedy, Thomas Jaecklin, Andrew McKibben, Elaine Chien, Marshall Baek, Pamela Vig, Cynthia Levy
BACKGROUND: Primary sclerosing cholangitis (PSC) is frequently associated with pruritus, which significantly impairs quality of life. Maralixibat is a selective ileal bile acid transporter (IBAT) inhibitor that lowers circulating bile acid (BA) levels and reduces pruritus in cholestatic liver diseases. This is the first proof-of-concept study of IBAT inhibition in PSC. METHODS: This open-label study evaluated the safety and tolerability of maralixibat ≤10 mg/d for 14 weeks in adults with PSC...
June 1, 2023: Hepatology Communications
#23
JOURNAL ARTICLE
C Fiorella Murillo Perez, Stephanie Ioannou, Iman Hassanally, Palak J Trivedi, Christophe Corpechot, Adriaan J van der Meer, Willem J Lammers, Pier Maria Battezzati, Keith D Lindor, Frederik Nevens, Kris V Kowdley, Tony Bruns, Nora Cazzagon, Annarosa Floreani, Andrew L Mason, Aliya Gulamhusein, Cyriel Y Ponsioen, Marco Carbone, Ana Lleo, Marlyn J Mayo, George N Dalekos, Nikolaos K Gatselis, Douglas Thorburn, Xavier Verhelst, Albert Parés, Maria-Carlota Londoño, Harry L A Janssen, Pietro Invernizzi, Raj Vuppalanchi, Gideon M Hirschfield, Bettina E Hansen, Cynthia Levy
BACKGROUND AND AIMS: Patients with primary biliary cholangitis (PBC) and insufficient response to ursodeoxycholic acid (UDCA), currently assessed after 1 year, are candidates for second-line therapy. The aims of this study are to assess biochemical response pattern and determine the utility of alkaline phosphatase (ALP) at six months as a predictor of insufficient response. METHODS: UDCA-treated patients in the GLOBAL PBC database with available liver biochemistries at one year were included...
May 8, 2023: Liver International: Official Journal of the International Association for the Study of the Liver
#24
JOURNAL ARTICLE
Victoria Mulcahy, Evaggelia Liaskou, Jose-Ezequiel Martin, Prasanti Kotagiri, Jonathan Badrock, Rebecca L Jones, Simon M Rushbrook, Stephen D Ryder, Douglas Thorburn, Simon D Taylor-Robinson, Graeme Clark, Heather J Cordell, Richard N Sandford, David E Jones, Gideon M Hirschfield, George F Mells
BACKGROUND AIMS: In patients with primary biliary cholangitis (PBC), the serum liver biochemistry measured during treatment with ursodeoxycholic acid-the UDCA response-accurately predicts long-term outcome. Molecular characterization of patients stratified by UDCA response can improve biological understanding of the high-risk disease, thereby helping to identify alternative approaches to disease-modifying therapy. In this study, we sought to characterize the immunobiology of the UDCA response using transcriptional profiling of peripheral blood mononuclear cell subsets...
April 1, 2023: Hepatology Communications
#25
JOURNAL ARTICLE
Natalie Uhlenbusch, Arpinder Bal, Boglárka Balogh, Annika Braun, Anja Geerts, Gideon Hirschfield, Maciej K Janik, Ansgar W Lohse, Piotr Milkiewicz, Mária Papp, Carine Poppe, Christoph Schramm, Bernd Löwe
BACKGROUND: Psychosocial support is a crucial component of adequate rare disease care, but to date psychosocial support needs of this patient population are insufficiently met. Within Q.RARE.LI, we strive to evaluate the effectiveness of a structured, transdiagnostic, and location-independent psychosocial support intervention in routine care of patients with rare autoimmune liver diseases in five countries and prepare its implementation. METHODS: Within an effectiveness-implementation hybrid trial, we aim to a) investigate the effectiveness of the intervention in routine care in five diverse healthcare systems and b) assess implementation outcomes, examine and prepare the implementation context, and develop country-specific implementation strategies...
March 24, 2023: BMC Psychiatry
#26
REVIEW
Amanda Ricciuto, Binita M Kamath, Gideon M Hirschfield, Palak J Trivedi
Autoimmune liver diseases are siloed into three syndromes that define clinical practice. These classifiers can, and are, challenged by variant presentations across all ages, something inevitable to disease definitions that rely on interpreting (inherently variable) semi-quantitative/qualitative clinical, laboratory, pathological or radiological findings. Furthermore this categorisation is premised on an ongoing absence of definable disease aetiologies. Clinicians thus encounter individuals with biochemical, serological, and histological manifestations that are common to both primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH), often labelled as 'PSC/AIH-overlap'...
August 2023: Journal of Hepatology
#27
REVIEW
Cynthia Levy, Michael Manns, Gideon Hirschfield
Primary biliary cholangitis (PBC) is an archetypal autoimmune disease. Chronic lymphocytic cholangitis is associated with interface hepatitis, ductopenia, cholestasis, and progressive biliary fibrosis. People living with PBC are frequently symptomatic, experiencing a quality-of-life burden dominated by fatigue, itch, abdominal pain, and sicca complex. Although the female predominance, specific serum autoantibodies, immune-mediated cellular injury, as well as genetic (HLA and non-HLA) risk factors, identify PBC as autoimmune, to date treatment has focused on cholestatic consequences...
July 2023: Clinical Gastroenterology and Hepatology
#28
Heather J Cordell, James J Fryett, Kazuko Ueno, Rebecca Darlay, Yoshihiro Aiba, Yuki Hitomi, Minae Kawashima, Nao Nishida, Seik-Soon Khor, Olivier Gervais, Yosuke Kawai, Masao Nagasaki, Katsushi Tokunaga, Ruqi Tang, Yongyong Shi, Zhiqiang Li, Brian D Juran, Elizabeth J Atkinson, Alessio Gerussi, Marco Carbone, Rosanna Asselta, Angela Cheung, Mariza de Andrade, Aris Baras, Julie Horowitz, Manuel A R Ferreira, Dylan Sun, David E Jones, Steven Flack, Ann Spicer, Victoria L Mulcahy, Jinyoung Byan, Younghun Han, Richard N Sandford, Konstantinos N Lazaridis, Christopher I Amos, Gideon M Hirschfield, Michael F Seldin, Pietro Invernizzi, Katherine A Siminovitch, Xiong Ma, Minoru Nakamura, George F Mells
No abstract text is available yet for this article.
January 11, 2023: Journal of Hepatology
#29
JOURNAL ARTICLE
Rozanne C de Veer, Maria C van Hooff, Christophe Corpechot, Douglas Thorburn, Pietro Invernizzi, Willem J Lammers, Harry L A Janssen, Pier M Battezzati, Frederik Nevens, Keith D Lindor, Annarosa Floreani, Cyriel Y Ponsioen, Marlyn J Mayo, Albert Parés, Andrew L Mason, Kris V Kowdley, Palak J Trivedi, Gideon M Hirschfield, Jorn C Goet, Tony Bruns, George N Dalekos, Nikolaos K Gatselis, Xavier Verhelst, Bettina E Hansen, Maren H Harms, Adriaan J van der Meer
BACKGROUND: Treatment of primary biliary cholangitis (PBC) can improve the GLOBE score. We aimed to assess the association between changes in the GLOBE score (ΔGLOBE) and liver transplantation (LT)-free survival in patients with PBC who were treated with ursodeoxycholic acid (UDCA). METHODS: Among UDCA-treated patients within the Global PBC cohort, the association between ΔGLOBE ( 0-1 : during first year of UDCA, 1-2 : during second year) and the risk of LT or death was assessed through Cox regression analyses...
December 8, 2022: American Journal of Gastroenterology
#30
JOURNAL ARTICLE
Carla F Murillo Perez, Alessio Gerussi, Palak J Trivedi, Christophe Corpechot, Adriaan J van der Meer, Pier Maria Battezzati, Keith D Lindor, Frederik Nevens, Kris V Kowdley, Tony Bruns, Nora Cazzagon, Annarosa Floreani, Atsushi Tanaka, Xiong Ma, Andrew L Mason, Aliya Gulamhusein, Cyriel Y Ponsioen, Marco Carbone, Ana Lleo, Marlyn J Mayo, George N Dalekos, Nikolaos K Gatselis, Douglas Thorburn, Xavier Verhelst, Albert Parés, Harry L A Janssen, Gideon M Hirschfield, Bettina E Hansen, Pietro Invernizzi, Willem J Lammers
BACKGROUND AND AIMS: The are geographic variations in the incidence and prevalence of primary biliary cholangitis (PBC). The aim was to explore whether clinical outcomes of patients within Western Europe differ according to geographical region. METHODS: Ursodeoxycholic acid-treated patients from European centers from the Global PBC database diagnosed from 1990 onwards were included. Patients with a time lag > 1 year from diagnosis to start of follow-up were excluded...
January 1, 2023: European Journal of Gastroenterology & Hepatology
#31
JOURNAL ARTICLE
Cynthia Levy, Stuart Kendrick, Christopher L Bowlus, Atsushi Tanaka, David Jones, Andreas E Kremer, Marlyn J Mayo, Nazneen Haque, Robyn von Maltzahn, Matthew Allinder, Brandon Swift, Megan M McLaughlin, Gideon M Hirschfield
BACKGROUND & AIMS: GLIMMER assessed dose-response, efficacy, and safety of linerixibat, an ileal bile acid transporter inhibitor in development for cholestatic pruritus associated with primary biliary cholangitis (PBC). METHODS: GLIMMER was a Phase 2b, multicenter, randomized, parallel-group study in adults with PBC and moderate-to-severe pruritus (≥4 on 0-10 numerical rating scale [NRS]). After 4 weeks of single-blind placebo, patients with NRS ≥3 were randomized (3:1) to double-blind linerixibat/placebo for 12 weeks (to week 16), followed by single-blind placebo (to week 20)...
November 4, 2022: Clinical Gastroenterology and Hepatology
#32
RANDOMIZED CONTROLLED TRIAL
Kris V Kowdley, Lisa Forman, Bertus Eksteen, Nadege Gunn, Vinay Sundaram, Charles Landis, Stephen A Harrison, Cynthia Levy, Alexander Liberman, Adrian M Di Bisceglie, Gideon M Hirschfield
INTRODUCTION: Primary sclerosing cholangitis (PSC) is a fibroinflammatory disease of the bile ducts leading to cirrhosis and hepatic decompensation. There are no approved pharmaceutical therapies for PSC. Berberine ursodeoxycholate (HTD1801) is an ionic salt of berberine and ursodeoxycholic acid with pleiotropic mechanisms of action. METHODS: An 18-week proof-of-concept study was conducted to assess the safety and efficacy of HTD1801 in PSC. This study had three 6-week periods: (i) a placebo-controlled period, (ii) a treatment extension period, and (iii) a randomized treatment withdrawal period...
November 1, 2022: American Journal of Gastroenterology
#33
REVIEW
Kristel K Leung, Gideon M Hirschfield
Primary biliary cholangitis (PBC) is a chronic immune-mediated liver disease characterized by a lymphocytic cholangitis, with subsequent cholestasis, progressive liver fibrosis, and ultimately complications arising from end-stage liver disease. Testing for autoantibodies is important in the diagnosis of PBC, as well as stratifying prognosis. This review focuses on the role of autoantibodies in the diagnosis of PBC, as well as the relationship between autoantibodies with pathophysiology and prognostication, along with a discussion regarding novel and other related disease autoantibodies...
November 2022: Clinics in Liver Disease
#34
JOURNAL ARTICLE
Christoph Schramm, Heiner Wedemeyer, Andrew Mason, Gideon M Hirschfield, Cynthia Levy, Kris V Kowdley, Piotr Milkiewicz, Ewa Janczewska, Elena Sergeevna Malova, Johanne Sanni, Phillip Koo, Jin Chen, Subhajit Choudhury, Lloyd B Klickstein, Michael K Badman, David Jones
Background & Aims: The safety, tolerability, and efficacy of the non-bile acid farnesoid X receptor agonist tropifexor were evaluated in a phase II, double-blind, placebo-controlled study as potential second-line therapy for patients with primary biliary cholangitis (PBC) with an inadequate ursodeoxycholic acid response. Methods: Patients were randomised (2:1) to receive tropifexor (30, 60, 90, or 150 μg) or matched placebo orally once daily for 28 days, with follow-up on Days 56 and 84...
November 2022: JHEP reports: innovation in hepatology
#35
JOURNAL ARTICLE
Carla F Murillo Perez, Tommy Ivanics, Marco P A W Claasen, Peter Yoon, David Wallace, Nazia Selzner, Gideon M Hirschfield, Bettina E Hansen, Gonzalo Sapisochin
BACKGROUND: To our knowledge, no analysis of data from liver transplantation registries exists in Canada. We aimed to describe temporal trends in the number of liver transplantation procedures, patient characteristics and posttransplantation outcomes for autoimmune liver diseases (AILDs) in Canada. METHODS: We used administrative data from the Canadian Organ Replacement Register, which contains liver transplantation information from 6 centres in Canada. This study included transplantation information from 5 of the centres, as liver transplantation procedures in children were not included...
September 2022: Canadian Journal of Surgery. Journal Canadien de Chirurgie
#36
RANDOMIZED CONTROLLED TRIAL
C Fiorella Murillo Perez, Holly Fisher, Shaun Hiu, Dorcas Kareithi, Femi Adekunle, Tracy Mayne, Elizabeth Malecha, Erik Ness, Adriaan J van der Meer, Willem J Lammers, Palak J Trivedi, Pier Maria Battezzati, Frederik Nevens, Kris V Kowdley, Tony Bruns, Nora Cazzagon, Annarosa Floreani, Andrew L Mason, Albert Parés, Maria-Carlota Londoño, Pietro Invernizzi, Marco Carbone, Ana Lleo, Marlyn J Mayo, George N Dalekos, Nikolaos K Gatselis, Douglas Thorburn, Xavier Verhelst, Aliya Gulamhusein, Harry L A Janssen, Rachel Smith, Steve Flack, Victoria Mulcahy, Michael Trauner, Christopher L Bowlus, Keith D Lindor, Christophe Corpechot, David Jones, George Mells, Gideon M Hirschfield, James Wason, Bettina E Hansen
BACKGROUND & AIMS: The Primary Biliary Cholangitis (PBC) Obeticholic Acid (OCA) International Study of Efficacy (POISE) randomized, double-blind, placebo-controlled trial demonstrated that OCA reduced biomarkers associated with adverse clinical outcomes (ie, alkaline phosphatase, bilirubin, aspartate aminotransferase, and alanine aminotransferase) in patients with PBC. The objective of this study was to evaluate time to first occurrence of liver transplantation or death in patients with OCA in the POISE trial and open-label extension vs comparable non-OCA-treated external controls...
December 2022: Gastroenterology
#37
JOURNAL ARTICLE
Rozanne C de Veer, Maren H Harms, Christophe Corpechot, Douglas Thorburn, Pietro Invernizzi, Harry L A Janssen, Pier M Battezzati, Frederik Nevens, Keith D Lindor, Annarosa Floreani, Cyriel Y Ponsioen, Marlyn J Mayo, Albert Parés, Andrew L Mason, Kris V Kowdley, Palak J Trivedi, Gideon M Hirschfield, Tony Bruns, George N Dalekos, Nikolaos K Gatselis, Xavier Verhelst, Willem J Lammers, Bettina E Hansen, Henk R van Buuren, Adriaan J van der Meer
BACKGROUND: After 1 year of ursodeoxycholic acid (UDCA), patients with primary biliary cholangitis (PBC) may have a normal GLOBE score despite high alkaline phosphatase (ALP) levels. AIM: To assess the association between ALP and liver transplantation (LT)-free survival according to the GLOBE score. METHODS: Among patients with a normal or elevated GLOBE score in the Global PBC cohort, the association between ALP after 1 year of UDCA therapy and the risk of LT/death was assessed...
September 22, 2022: Alimentary Pharmacology & Therapeutics
#38
JOURNAL ARTICLE
Nadir Abbas, Emma L Culver, Douglas Thorburn, Neil Halliday, Hannah Crothers, Jessica K Dyson, April Phaw, Richard Aspinall, Salim I Khakoo, Yiannis Kallis, Belinda Smith, Imran Patanwala, Anne McCune, Chenchu R Chimakurthi, Vinod Hegade, Michael Orrell, Rebecca Jones, George Mells, Colette Thain, Robert-Mitchell Thain, David Jones, Gideon Hirschfield, Palak J Trivedi
BACKGROUND AND AIMS: One-third of patients with primary biliary cholangitis (PBC) inadequately respond to ursodeoxycholic acid (UDCA). Our aim was to assemble national, real-world data on the effectiveness of obeticholic acid (OCA) as a second-line treatment, alongside non-licensed therapy with fibric acid derivatives (bezafibrate/fenofibrate). METHODS: This was a nationwide observational cohort study conducted from August 2017 until June 2021. RESULTS: We accrued data from 457 patients; 349 treated with OCA and 108 with fibric acid derivatives...
August 8, 2022: Clinical Gastroenterology and Hepatology
#39
JOURNAL ARTICLE
Christophe Corpechot, Fabrice Carrat, Farid Gaouar, Frederic Chau, Gideon Hirschfield, Aliya Gulamhusein, Aldo J Montano-Loza, Ellina Lytvyak, Christoph Schramm, Albert Pares, Ignasi Olivas, John E Eaton, Karim T Osman, George Dalekos, Nikolaos Gatselis, Frederik Nevens, Nora Cazzagon, Alessandra Zago, Francesco Paolo Russo, Nadir Abbas, Palak Trivedi, Douglas Thorburn, Francesca Saffioti, Laszlo Barkai, Davide Roccarina, Vicenza Calvaruso, Anna Fichera, Adèle Delamarre, Esli Medina-Morales, Alan Bonder, Vilas Patwardhan, Cristina Rigamonti, Marco Carbone, Pietro Invernizzi, Laura Cristoferi, Adriaan van der Meer, Rozanne de Veer, Ehud Zigmond, Eyal Yehezkel, Andreas E Kremer, Ansgar Deibel, Jérôme Dumortier, Tony Bruns, Karsten Große, Georges-Philippe Pageaux, Aaron Wetten, Jessica Dyson, David Jones, Olivier Chazouillères, Bettina Hansen, Victor de Lédinghen
BACKGROUND & AIMS: Liver stiffness measurement (LSM) by vibration-controlled transient elastography (VCTE) has been shown to predict outcomes of patients with primary biliary cholangitis (PBC) in small-size studies. We aimed to validate the prognostic value of LSM in a large cohort study. METHODS: We performed an international, multicentre, retrospective follow-up study of 3,985 patients with PBC seen at 23 centres in 12 countries. Eligibility criteria included at least 1 reliable LSM by VCTE and a follow-up ≥ 1 year...
December 2022: Journal of Hepatology
#40
JOURNAL ARTICLE
David E J Jones, Aaron Wetten, Ben Barron-Millar, Laura Ogle, George Mells, Steven Flack, Richard Sandford, John Kirby, Jeremy Palmer, Sophie Brotherston, Laura Jopson, John Brain, Graham R Smith, Steve Rushton, Rebecca Jones, Simon Rushbrook, Douglas Thorburn, Stephen D Ryder, Gideon Hirschfield, Jessica K Dyson
BACKGROUND: Uncertainty exists about how best to identify primary biliary cholangitis (PBC) patients who would benefit from second-line therapy. Existing, purely clinical, ursodeoxycholic acid (UDCA) response criteria accept degrees of liver biochemistry abnormality in responding patients, emerging data, however, suggest that any degree of ongoing abnormality may, in fact, be associated with an increased risk of adverse outcomes. This cohort study explores the link between response status, the biology of high-risk disease and its implications for clinical practice...
June 2022: EBioMedicine
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