Unmet clinical need in autoimmune liver diseases

被引:54
作者
Dyson, Jessica K. [1 ]
Webb, Gwilym [2 ,3 ]
Hirschfield, Gideon M. [2 ,3 ,4 ]
Lohse, Ansgar [5 ]
Beuers, Ulrich [6 ]
Lindor, Keith [7 ]
Jones, David E. J. [1 ,4 ,8 ]
机构
[1] Freeman Rd Hosp, Liver Unit, Newcastle Upon Tyne, Tyne & Wear, England
[2] Univ Birmingham, Liver Res Ctr, Birmingham B15 2TT, W Midlands, England
[3] Univ Birmingham, NIHR Biomed Res Unit, Birmingham B15 2TT, W Midlands, England
[4] UK PBC Res Consortium, Newcastle Upon Tyne, Tyne & Wear, England
[5] Univ Klinikum Eppendorf, Hamburg, Germany
[6] Amsterdam Med Ctr, Dept Gastroenterol & Hepatol, Amsterdam, Netherlands
[7] Arizona State Univ, Med Ctr, Tempe, AZ 85287 USA
[8] Newcastle Univ, Inst Cellular Med, Newcastle Upon Tyne NE2 4HH, Tyne & Wear, England
基金
英国医学研究理事会;
关键词
Unmet clinical need; Autoimmune liver diseases; Primary biliary cirrhosis; Primary sclerosing cholangitis; Autoimmune hepatitis; IgG4; disease; Stratified care delivery; PRIMARY BILIARY-CIRRHOSIS; PRIMARY SCLEROSING CHOLANGITIS; DOSE URSODEOXYCHOLIC ACID; URINARY-TRACT-INFECTION; QUALITY-OF-LIFE; TRANSIENT ELASTOGRAPHY; BIOCHEMICAL RESPONSE; ANTIMITOCHONDRIAL ANTIBODIES; ANTICENTROMERE ANTIBODIES; INCREASED PREVALENCE;
D O I
10.1016/j.jhep.2014.09.010
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
Despite recent advances in understanding and treatment, there remain significant areas of unmet clinical need in each of the autoimmune liver diseases (AILDs). The evolving research landscape and emerging large patient cohorts are creating unique opportunities to translate science into new therapies and care pathways, with the potential to significantly improve the lives of AILD patients. However, the areas of unmet need represent real challenges, which need to be addressed, if this vision is to be realised. This review describes the areas of unmet need in AILD in adults relating to diagnostic and prognostic assessment, primary therapy, symptom management, trial design and delivery, and structured care delivery, with the aim of focusing future research prioritisation. (C) 2014 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.
引用
收藏
页码:208 / 218
页数:11
相关论文
共 106 条
[1]   Improvement of serum alkaline phosphatase to <1.5 upper limit of normal predicts better outcome and reduced risk of cholangiocarcinoma in primary sclerosing cholangitis [J].
Al Mamari, Said ;
Djordjevic, Jelena ;
Halliday, John S. ;
Chapman, Roger W. .
JOURNAL OF HEPATOLOGY, 2013, 58 (02) :329-334
[2]   Increased prevalence of primary biliary cirrhosis near superfund toxic waste sites [J].
Ala, A ;
Stanca, CM ;
Bu-Ghanim, M ;
Ahmado, I ;
Branch, AD ;
Schiano, TD ;
Odin, JA ;
Bach, N .
HEPATOLOGY, 2006, 43 (03) :525-531
[3]   International Autoimmune Hepatitis Group Report:: review of criteria for diagnosis of autoimmune hepatitis [J].
Alvarez, E ;
Berg, PA ;
Bianchi, FB ;
Bianchi, L ;
Burroughs, AK ;
Cancado, EL ;
Chapman, RW ;
Cooksley, WGE ;
Czaja, AJ ;
Desmet, VJ ;
Donaldson, RT ;
Eddleston, ALWF ;
Fainboim, L ;
Heathcote, J ;
Homberg, JC ;
Hoofnagle, JH ;
Kakumu, S ;
Krawitt, EL ;
Mackay, IR ;
MacSween, RNM ;
Maddrey, WC ;
Manns, MP ;
McFarlane, IG ;
zum Büschenfelde, KHM ;
Mieli-Vergani, G ;
Nakanuma, Y ;
Nishioka, M ;
Penner, E ;
Porta, G ;
Portmann, BC ;
Reed, WD ;
Rodes, J ;
Schalm, SW ;
Scheuer, PJ ;
Schrumpf, E ;
Seki, T ;
Toda, G ;
Tsuji, T ;
Tygstrup, N ;
Vergani, D ;
Zeniya, M .
JOURNAL OF HEPATOLOGY, 1999, 31 (05) :929-938
[4]   Oral budesonide in the treatment of patients with primary biliary cirrhosis with a suboptimal response to ursodeoxycholic acid [J].
Angulo, P ;
Jorgensen, RA ;
Keach, JC ;
Dickson, ER ;
Smith, C ;
Lindor, KD .
HEPATOLOGY, 2000, 31 (02) :318-323
[5]   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
[6]   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
[7]   Fatigue in patients with primary sclerosing cholangitis [J].
Björnsson, E ;
Simren, M ;
Olsson, R ;
Chapman, RW .
SCANDINAVIAN JOURNAL OF GASTROENTEROLOGY, 2004, 39 (10) :961-968
[8]   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
[9]   Features of autoimmune hepatitis in primary sclerosing cholangitis: An evaluation of 114 primary sclerosing cholangitis patients according to a scoring system for the diagnosis of autoimmune hepatitis [J].
Boberg, KM ;
Fausa, O ;
Haaland, T ;
Holter, E ;
Mellbye, OJ ;
Spurkland, A ;
Schrumpf, E .
HEPATOLOGY, 1996, 23 (06) :1369-1376
[10]   PBC MANAGEMENT AMONG CLINICIANS: GAPS IN CLINICAL COMPETENCE AND PRACTICE PERFORMANCE SURVEY FINDINGS [J].
Bodenheimer, H. C., Jr. ;
Jones, D. E. J. ;
Peterson, P. ;
Rudell, E. .
JOURNAL OF HEPATOLOGY, 2014, 60 (01) :S201-S201