Literature DB >> 29067151

Primary sclerosing cholangitis and the management of uncertainty and complexity.

Katherine Arndtz1,2, Gideon M Hirschfield1,2.   

Abstract

Primary sclerosing cholangitis (PSC) is a rare chronic autoimmune disease with no effective therapy and a large unmet need for new treatments. Patients require significant healthcare resources over their lifetime with high rates of hospital admission, malignancy, liver transplantation and death. As a rare disease, expertise in management can be limited to large referral liver transplant programmes, and even then there is frequently variation in practice. In this case-based review, we aim to discuss common clinical dilemmas encountered by clinicians managing patients with PSC and address related competencies in the 2010 Gastroenterology Curriculum.

Entities:  

Keywords:  autoimmune liver disease; cholestatic liver diseases; inflammatory bowel diseases; orthotopic liver transplantation; primarysclerosing colangitis

Year:  2017        PMID: 29067151      PMCID: PMC5641853          DOI: 10.1136/flgastro-2017-100815

Source DB:  PubMed          Journal:  Frontline Gastroenterol        ISSN: 2041-4137


  10 in total

1.  EASL Clinical Practice Guidelines: management of cholestatic liver diseases.

Authors: 
Journal:  J Hepatol       Date:  2009-06-06       Impact factor: 25.083

2.  Primary sclerosing cholangitis: 50 years of a gut-liver relationship and still no love?

Authors:  Tom H Karlsen
Journal:  Gut       Date:  2016-07-11       Impact factor: 23.059

3.  Patient Age, Sex, and Inflammatory Bowel Disease Phenotype Associate With Course of Primary Sclerosing Cholangitis.

Authors:  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
Journal:  Gastroenterology       Date:  2017-03-06       Impact factor: 22.682

4.  International Autoimmune Hepatitis Group Report: review of criteria for diagnosis of autoimmune hepatitis.

Authors:  F Alvarez; P A Berg; F B Bianchi; L Bianchi; A K Burroughs; E L Cancado; R W Chapman; W G Cooksley; A J Czaja; V J Desmet; P T Donaldson; A L Eddleston; L Fainboim; J Heathcote; J C Homberg; J H Hoofnagle; S Kakumu; E L Krawitt; I R Mackay; R N MacSween; W C Maddrey; M P Manns; I G McFarlane; K H Meyer zum Büschenfelde; M Zeniya
Journal:  J Hepatol       Date:  1999-11       Impact factor: 25.083

5.  The Pathogenesis of Autoimmune Liver Disease.

Authors:  Katherine Arndtz; Gideon M Hirschfield
Journal:  Dig Dis       Date:  2016-05-11       Impact factor: 2.404

6.  Population-based epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis.

Authors:  Kirsten Boonstra; Rinse K Weersma; Karel J van Erpecum; Erik A Rauws; B W Marcel Spanier; Alexander C Poen; Karin M van Nieuwkerk; Joost P Drenth; Ben J Witteman; Hans A Tuynman; Anton H Naber; Paul J Kingma; Henk R van Buuren; Bart van Hoek; Frank P Vleggaar; Nan van Geloven; Ulrich Beuers; Cyriel Y Ponsioen
Journal:  Hepatology       Date:  2013-10-17       Impact factor: 17.425

Review 7.  Primary sclerosing cholangitis.

Authors:  Gideon M Hirschfield; Tom H Karlsen; Keith D Lindor; David H Adams
Journal:  Lancet       Date:  2013-06-28       Impact factor: 79.321

Review 8.  Surrogate endpoints for clinical trials in primary sclerosing cholangitis: Review and results from an International PSC Study Group consensus process.

Authors:  Cyriel Y Ponsioen; Roger W Chapman; Olivier Chazouillères; Gideon M Hirschfield; Tom H Karlsen; Ansgar W Lohse; Massimo Pinzani; Erik Schrumpf; Michael Trauner; Gregory J Gores
Journal:  Hepatology       Date:  2015-12-23       Impact factor: 17.425

Review 9.  Unmet clinical need in autoimmune liver diseases.

Authors:  Jessica K Dyson; Gwilym Webb; Gideon M Hirschfield; Ansgar Lohse; Ulrich Beuers; Keith Lindor; David E J Jones
Journal:  J Hepatol       Date:  2014-09-16       Impact factor: 25.083

10.  Genome-wide association study of primary sclerosing cholangitis identifies new risk loci and quantifies the genetic relationship with inflammatory bowel disease.

Authors:  Sun-Gou Ji; Brian D Juran; Sören Mucha; Trine Folseraas; Luke Jostins; Espen Melum; Natsuhiko Kumasaka; Elizabeth J Atkinson; Erik M Schlicht; Jimmy Z Liu; Tejas Shah; Javier Gutierrez-Achury; Kirsten M Boberg; Annika Bergquist; Severine Vermeire; Bertus Eksteen; Peter R Durie; Martti Farkkila; Tobias Müller; Christoph Schramm; Martina Sterneck; Tobias J Weismüller; Daniel N Gotthardt; David Ellinghaus; Felix Braun; Andreas Teufel; Mattias Laudes; Wolfgang Lieb; Gunnar Jacobs; Ulrich Beuers; Rinse K Weersma; Cisca Wijmenga; Hanns-Ulrich Marschall; Piotr Milkiewicz; Albert Pares; Kimmo Kontula; Olivier Chazouillères; Pietro Invernizzi; Elizabeth Goode; Kelly Spiess; Carmel Moore; Jennifer Sambrook; Willem H Ouwehand; David J Roberts; John Danesh; Annarosa Floreani; Aliya F Gulamhusein; John E Eaton; Stefan Schreiber; Catalina Coltescu; Christopher L Bowlus; Velimir A Luketic; Joseph A Odin; Kapil B Chopra; Kris V Kowdley; Naga Chalasani; Michael P Manns; Brijesh Srivastava; George Mells; Richard N Sandford; Graeme Alexander; Daniel J Gaffney; Roger W Chapman; Gideon M Hirschfield; Mariza de Andrade; Simon M Rushbrook; Andre Franke; Tom H Karlsen; Konstantinos N Lazaridis; Carl A Anderson
Journal:  Nat Genet       Date:  2016-12-19       Impact factor: 38.330

  10 in total
  4 in total

Review 1.  Update in the Care and Management of Patients with Primary Sclerosing Cholangitis.

Authors:  Mai Sedki; Cynthia Levy
Journal:  Curr Gastroenterol Rep       Date:  2018-06-09

2.  Bridging the Gap Between Competencies and Uncertainties in Postgraduate Training in Family Medicine: Results and Psychometric Properties of a Self-Assessment Questionnaire.

Authors:  Kristina Flägel; Marie-Therese Müller; Katja Goetz; Elisabeth Flum; Simon Schwill; Jost Steinhäuser
Journal:  Adv Med Educ Pract       Date:  2022-07-04

3.  Quality of life (QoL) for people with primary sclerosing cholangitis (PSC): a pragmatic strategy for identifying relevant QoL issues for rare disease.

Authors:  Elena Marcus; Patrick Stone; Douglas Thorburn; Martine Walmsley; Bella Vivat
Journal:  J Patient Rep Outcomes       Date:  2022-07-15

4.  Quality of life in primary sclerosing cholangitis: a systematic review.

Authors:  Elena Marcus; Paddy Stone; Anna-Maria Krooupa; Douglas Thorburn; Bella Vivat
Journal:  Health Qual Life Outcomes       Date:  2021-03-20       Impact factor: 3.186

  4 in total

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