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
[61]
Autoimmune hepatitis with incidental histologic features of bile duct injury [J].
Czaja, AJ ;
Carpenter, HA .
HEPATOLOGY, 2001, 34 (04) :659-665
[62]
Cholestatic Phenotypes of Autoimmune Hepatitis [J].
Czaja, Albert J. .
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY, 2014, 12 (09) :1430-1438
[63]
Pediatric-onset primary biliary cirrhosis [J].
Dahlan, Y ;
Smith, L ;
Simmonds, D ;
Jewell, LD ;
Wanless, I ;
Heathcote, EJ ;
Bain, VG .
GASTROENTEROLOGY, 2003, 125 (05) :1476-1479
[64]
Large-Scale Characterization Study of Patients With Antimitochondrial Antibodies but Nonestablished Primary Biliary Cholangitis [J].
Dahlqvist, Geraldine ;
Gaouar, Farid ;
Carrat, Fabrice ;
Meurisse, Sofia ;
Chazouilleres, Olivier ;
Poupon, Raoul ;
Johanet, Catherine ;
Corpechot, Christophe .
HEPATOLOGY, 2017, 65 (01) :152-163
[65]
DATTA DV, 1966, GASTROENTEROLOGY, V50, P323
[67]
Cholestasis-induced pruritus treated with ultraviolet B phototherapy: An observational case series study [J].
Decock, Sofie ;
Roelandts, Rik ;
Van Steenbergen, Werner ;
Laleman, Wim ;
Cassiman, David ;
Verslype, Chris ;
Fevery, Johan ;
Van Pelt, Jos ;
Nevens, Frederik .
JOURNAL OF HEPATOLOGY, 2012, 57 (03) :637-641
[68]
Effect of ursodeoxycholic acid on bile acid profiles and intestinal detoxification machinery in primary biliary cirrhosis and health [J].
Dilger, Karin ;
Hohenester, Simon ;
Winkler-Budenhofer, Ursula ;
Bastiaansen, Barbara A. J. ;
Schaap, Frank G. ;
Rust, Christian ;
Beuers, Ulrich .
JOURNAL OF HEPATOLOGY, 2012, 57 (01) :133-140
[69]
Patients With Autoimmune Hepatitis Who Have Antimitochondrial Antibodies Need Long-term Follow-up to Detect Late Development of Primary Biliary Cirrhosis [J].
Dinani, Amreen M. ;
Fischer, Sandra E. ;
Mosko, Jeff ;
Guindi, Maha ;
Hirschfield, Gideon M. .
CLINICAL GASTROENTEROLOGY AND HEPATOLOGY, 2012, 10 (06) :682-684
[70]
The inter-relationship of symptom severity and quality of life in 2055 patients with primary biliary cholangitis [J].
Dyson, J. K. ;
Wilkinson, N. ;
Jopson, L. ;
Mells, G. ;
Bathgate, A. ;
Heneghan, M. A. ;
Neuberger, J. ;
Hirschfield, G. M. ;
Ducker, S. J. ;
Sandford, R. ;
Alexander, G. ;
Stocken, D. ;
Jones, D. E. J. .
ALIMENTARY PHARMACOLOGY & THERAPEUTICS, 2016, 44 (10) :1039-1050