EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis

被引:1165
作者
Hirschfield, Gideon M. [1 ]
Beuers, Ulrich [1 ]
Corpechot, Christophe [1 ]
Invernizzi, Pietro [1 ]
Jones, David [1 ]
Marzioni, Marco [1 ]
机构
[1] EASL, EASL Bldg,7 Rue Daubin, CH-1203 Geneva, Switzerland
基金
英国医学研究理事会;
关键词
Cholestasis; Guidelines; Care pathway; Liver; QUALITY-OF-LIFE; RANDOMIZED CONTROLLED-TRIALS; ACID-TREATED PATIENTS; PRIMARY SCLEROSING CHOLANGITIS; LIVER FIBROSIS PROGRESSION; EARLY BIOCHEMICAL RESPONSE; ORAL NALTREXONE TREATMENT; PLACEBO-CONTROLLED TRIAL; LONG-TERM PROGNOSIS; URSODEOXYCHOLIC-ACID;
D O I
10.1016/j.jhep.2017.03.022
中图分类号
R57 [消化系及腹部疾病];
学科分类号
100201 [内科学];
摘要
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. Pharmacologic approaches in practice, to reduce the impact of the progressive nature of disease, currently include licensed therapies (ursodeoxycholic acid and obeticholic acid) and off-label therapies (fibric acid derivatives, budesonide). These clinical practice guidelines summarise the evidence for the importance of a structured, life-long and individualised, approach to the care of patients with PBC, providing a framework to help clinicians diagnose and effectively manage patients. (C) 2017 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.
引用
收藏
页码:145 / 172
页数:28
相关论文
共 260 条
[21]
Changing nomenclature for PBC: From 'cirrhosis' to 'cholangitis' [J].
Beuers, Ulrich ;
Gershwin, M. Eric ;
Gish, Robert G. ;
Invernizzi, Pietro ;
Jones, David E. J. ;
Lindor, Keith ;
Ma, Xiong ;
Mackay, Ian R. ;
Pares, Albert ;
Tanaka, Atsushi ;
Vierling, John M. ;
Poupon, Raoul .
JOURNAL OF HEPATOLOGY, 2015, 63 (05) :1285-1287
[22]
New paradigms in the treatment of hepatic cholestasis: From UDCA to FXR, PXR and beyond [J].
Beuers, Ulrich ;
Trauner, Michael ;
Jansen, Peter ;
Poupon, Raoul .
JOURNAL OF HEPATOLOGY, 2015, 62 :S25-S37
[23]
Pruritus in Cholestasis: Facts and Fiction [J].
Beuers, Ulrich ;
Kremer, Andreas E. ;
Bolier, Ruth ;
Elferink, Ronald P. J. Oude .
HEPATOLOGY, 2014, 60 (01) :399-407
[24]
The Binary HCO3- Umbrella: A Unifying Hypothesis on Pathogenetic and Therapeutic Aspects of Fibrosing Cholangiopathies [J].
Beuers, Ulrich ;
Hohenester, Simon ;
Wenniger, Lucas J. Maillette de Buy ;
Kremer, Andreas E. ;
Jansen, Peter L. M. ;
Elferink, Ronald P. J. Oude .
HEPATOLOGY, 2010, 52 (04) :1489-1496
[25]
EASL Clinical Practice Guidelines: Management of cholestatic liver diseases [J].
Beuers, Ulrich ;
Boberg, Kirsten M. ;
Chapman, Roger W. ;
Chazouilleres, Olivier ;
Invernizzi, Pietro ;
Jones, David E. J. ;
Lammert, Frank ;
Pares, Albert ;
Trauner, Michael .
JOURNAL OF HEPATOLOGY, 2009, 51 (02) :237-267
[26]
Genetics and Epigenetics of Primary Biliary Cirrhosis [J].
Bianchi, Ilaria ;
Carbone, Marco ;
Lleo, Ana ;
Invernizzi, Pietro .
SEMINARS IN LIVER DISEASE, 2014, 34 (03) :255-264
[27]
The X chromosome and immune associated genes [J].
Bianchi, Ilaria ;
Lleo, Ana ;
Gershwin, M. Eric ;
Invernizzi, Pietro .
JOURNAL OF AUTOIMMUNITY, 2012, 38 (2-3) :J187-J192
[28]
The role of psychological factors in the fatigue of primary biliary cirrhosis [J].
Blackburn, Peter ;
Freeston, Mark ;
Baker, Christine R. ;
Jones, David E. J. ;
Newton, Julia L. .
LIVER INTERNATIONAL, 2007, 27 (05) :654-661
[29]
Overlap syndromes: The International Autoimmune Hepatitis Group (IAIHG) position statement on a controversial issue [J].
Boberg, Kirsten Muri ;
Chapman, Roger W. ;
Hirschfield, Gideon M. ;
Lohse, Ansgar W. ;
Manns, Michael P. ;
Schrumpf, Erik .
JOURNAL OF HEPATOLOGY, 2011, 54 (02) :374-385
[30]
Rising incidence and prevalence of primary biliary cirrhosis: a large population-based study [J].
Boonstra, Kirsten ;
Kunst, Anton E. ;
Stadhouders, Paul H. ;
Tuynman, Hans A. ;
Poen, Alexander C. ;
van Nieuwkerk, Karin. M. J. ;
Witteman, Ellen M. ;
Hamann, Dorte ;
Witteman, Ben J. ;
Beuers, Ulrich ;
Ponsioen, Cyriel Y. .
LIVER INTERNATIONAL, 2014, 34 (06) :E31-E38